Paeds

Question 1

Gross motor milestones

3 months

Answer 1

Little or no head lag on being pulled to sit

Lying on abdomen, good head control

Held sitting, lumbar curve

Question 2

Gross motor milestones

6 months

Answer 2

Lying on abdomen arms extended

Lying on back, lifts and grasps feet

Pulls self to sitting

Held sitting, back straight

Rolls front to back

Question 3

Gross motor milestones

7-8m

Answer 3

Sits without support

(Refer at 12m)

Question 4

Gross motor milestones

9 months

Answer 4

Pulls to standing

Crawls

Question 5

Gross motor milestones

12m

Answer 5

Cruises

Walks with one hand held

Question 6

Gross motor milestones

13-15m

Answer 6

Walks unsupported

(Refer at 18m)

Question 7

Gross motor milestones

2y

Answer 7

Runs

Walks upstairs and downstairs holding onto rail

Question 8

Gross motor milestones

3y

Answer 8

Rides a tricycle using pedals

Walks up stairs without holding a rail

Question 9

Gross motor milestones

4y

Answer 9

Hops on one leg

Question 10

Pyloric stenosis presentation:

Answer 10

Pyloric stenosis typically presents in the second to fourth weeks of life with vomiting, although rarely may present later at up to four months. It is caused by hypertrophy of the circular muscles of the pylorus

Question 11

Features of Py Sten

Answer 11

‘projectile’ vomiting, typically 30 minutes after a feed

constipation and dehydration may also be present

a palpable mass may be present in the upper abdomen

hypochloraemic, hypokalaemic alkalosis due to persistent vomiting

Question 12

Dx of py sten

Answer 12

USS

Question 13

Ramstedt pylorotomy

Answer 13

Used in management of py sten

Excision of the hypertrophied circular muscles of the pylorus

Question 14

Def intussuception

Answer 14

Intussusception describes the invagination of one portion of bowel into the lumen of the adjacent bowel, most commonly around the ileo-caecal region.

Intussusception usually affects infants between 6-18 months old. Boys are affected twice as often as girls

Question 15

Features of intussuception

Answer 15

paroxysmal abdominal colic pain

during paroxysm the infant will characteristically draw their knees up and turn pale

vomiting

blood stained stool - ‘red-currant jelly’

sausage-shaped mass in the right lower quadrant

Question 16

Ix intussuception

Answer 16

USS

Question 17

Mx of intussuception

Answer 17

Air insuffation under radiological control

If the child has signs of peritonitis or the air insufflation fails, Sx

Question 18

A 2-month-old boy is brought to the afternoon surgery by his mother. Since the morning he has been taking reduced feeds and has been ‘not his usual self’. On examination the baby appears well but has a temperature of 38.7ºC. What is the most appropriate management?

Advise regarding antipyretics, to see if not settling

IM benzylpenicillin

Advise regarding antipyretics, booked appointment for next day

Admit to hospital

Empirical amoxicillin for 7 days

Answer 18

Any child less than 3 months old with a temperature > 38ºC is regarded as a ‘red’ feature in the new NICE guidelines, warranting urgent referral to a paediatrician. Although many experienced GPs may choose not to strictly follow such advice it is important to be aware of recent guidelines for the exam

Question 19

Assessment of febrile children?

Answer 19

T: electronic thermometer in the axilla if <4w or with infra-red tympanic thermometer

HR

RR

CRT

Signs of dehydration: skin turgor

Question 20

What are the categroies on the feverish illness guidelines

Answer 20

Colour

Activity

Respiratory

Circulation and hydration

Other

Question 21

Mx of child at “green” on risk stratificiation for feverish illness?

Answer 21

Managed at home with appropriate care advice, including when to seek further help

Question 22

Mx of child at “amber” on risk stratificiation for feverish illness?

Answer 22

Safety net or refer to paediatric specialist for further assessment

Safety net: verbal/written info about warning symptoms and how to access further care

Question 23

Mx of child at “red” on risk stratificiation for feverish illness?

Answer 23

Admit to hospital

Question 24

Key points for Mx of fever in child

Answer 24

Oral antibiotics should not be prescribed without identification of an apparent source of fever

Question 25

CXR in ?pneumonia in children?

Answer 25

Not routinely performed

Question 26

Green Colour

Answer 26

Normal

Question 27

Green Activity

Answer 27

Responds normally to social cures Content/smiles Stays awake or wakens quickly Strong normal cry/not crying

Question 28

Green Circulation and hydration

Answer 28

Normal skin and eyes Moist mucous membranes

Question 29

Important for Green risk stratification

Answer 29

No amber or red signs present

Question 30

Amber Coour

Answer 30

Pallor reported by patient/carer

Question 31

Amber Activity

Answer 31

Not responding to social cues normally No smiles Wakes only with prolonged stimulation Decreased activity

Question 32

Amber Respiratory

Answer 32

Nasal flaring Tachypnoea \>50 breaths/m aged 6-12 \>40 breats/minute age, 12m Oxygen saturation \<95% on air Crackles in chest

Question 33

Amber Circulation

Answer 33

Tachycardia 12m: \>160bpm 12-24m \>150bpm 2-5y \>140 CRT \>3 Dry mucous membranes Poor feeding in infants Reduced urine output

Question 34

Amber Other things of note

Answer 34

3-6m \>39 deg temperature Fever for \>5d Rigors Swelling of a limb or joint Non-weight bearing limb/not using an extremity

Question 35

Red Colour

Answer 35

Pale/mottled/ashen/blue

Question 36

Red Activity

Answer 36

No response to social cues Appears ill to healthcare professional Does not wake or does not stay awake Weak, high-pitched or continuous cry

Question 37

Red Respiratory

Answer 37

Grunting Tachypnoea: RR \>60 Moderate or severe chest indrawing

Question 38

Red Circulation

Answer 38

Reduced skin turgor

Question 39

Red Other features

Answer 39

Age \<3m, T \>38 Non blanching rash Bulging fontanelle Neck stiffness Status epilepticus Focal neurological signs Foal seizures

Question 40

Tachypnoea 6-12m

Answer 40

\>50

Question 41

Tachypnoea \>12m

Answer 41

\>40

Question 42

Tachycardia \<12m

Answer 42

\>160bpm

Question 43

Tachycardia 12-24m

Answer 43

\>150bpm

Question 44

Tachycardia 2-5y

Answer 44

\>140bpm

Question 45

A 3-year-old girl is brought in by her mother. Her mother reports that she has been eating less and refusing food for the past few weeks. Despite this her mother has noticed that her abdomen is distended and she has developed a 'beer belly'. For the past year she has opened her bowels around once every other day, passing a stool of 'normal' consistency. There are no urinary symptoms. On examination she is on the 50th centile for height and weight. Her abdomen is soft but slightly distended and a non-tender ballotable mass can be felt on the left side. Her mother has tried lactulose but there has no significant improvement. What is the most appropriate next step in management? ## Footnote Switch to polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) and review in two weeks Speak to a local paediatrician Reassure normal findings and advise Health Visitor review to improve oral intake Prescribe a Microlax enema Continue lactulose and add ispaghula husk sachets

Answer 45

The history of constipation is not particularly convincing. A child passing a stool of normal consistency every other day is within the boundaries of normal. The key point to this question is recognising the abnormal examination finding - a ballotable mass associated with abdominal distension. Whilst an adult with such a 'red flag' symptom/sign would be fast-tracked it is more appropriate to speak to a paediatrician to determine the best referral pathway, which would probably be clinic review the same week. Wilms' tumour

Question 46

WIlm's tumour

Answer 46

Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old.

Question 47

Features of Wilm's tumour

Answer 47

Abdominal mass (most common PC) Painless haematuria Flank pain Anorexia, fever Unilateral in 95% Mest found in 20%

Question 48

Wilm's associated with

Answer 48

Beckwith-Wiedemann syndrome As part of WAGR syndrome Hemihypertrophy 1/3rd associated with a lof mutation in WT1 on chromosome 11

Question 49

Beckwith-Wiedemann syndrome

Answer 49

Beckwith-Wiedemann syndrome is a condition that affects many parts of the body. It is classified as an overgrowth syndrome, which means that affected infants are considerably larger than normal (macrosomia) and tend to be taller than their peers during childhood. Growth begins to slow by about age 8, and adults with this condition are not unusually tall. In some children with Beckwith-Wiedemann syndrome, specific parts of the body on one side or the other may grow abnormally large, leading to an asymmetric or uneven appearance. This unusual growth pattern, which is known as hemihyperplasia, usually becomes less apparent over time.

Question 50

WAGR Syndrome

Answer 50

WAGR syndrome is a rare geneticsyndrome in which affected children are predisposed to develop Wilms tumour (a tumour of the kidneys), Aniridia (absence of the coloured part of the eye, the iris),Genitourinary anomalies, and Retardation.[1]The G is sometimes instead given as "gonadoblastoma," since the genitourinary anomalies are tumours of the gonads (testes or ovaries).[2] A subset of WAGR syndrome patients shows severe childhood obesity; the acronymWAGRO (O for obesity) has been used to describe this category.[3] The condition results from a deletion on chromosome 11 resulting in the loss of severalgenes. As such, it is one of the best studied examples of a condition caused by loss of neighbouring (contiguous) genes.[3]

Question 51

Mx of Wilm's

Answer 51

Management nephrectomy chemotherapy radiotherapy if advanced disease prognosis: good, 80% cure rate

Question 52

Histological features of WIlms

Answer 52

Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell blastomatous tissues resembling the metanephric blastema

Question 53

Features of pertussis

Answer 53

Caused by Bordetella pertussis 10-14d incubation Infants rountely immunised at 2,3,4m and 3-5y. Pregnant women also immunised

Question 54

Clinical features of pertussis

Answer 54

Coughing bouts: usually worse at night and after feeding, may be ended by vomiting and associated central cyanosis inspiratory whoop (not always present), caused by forced inspriration against a closed glottis Persistent coughing may cause subconjunctival haemorrhages or anorexia, leading to syncope and seizures Symptoms may last 10-14w and tend to be more severe in infants **Lymphocytosis**

Question 55

Dx of pertussis

Answer 55

Per nasal swab culture for B. pertussis PCR and serology may also be used

Question 56

Mx of pertussis

Answer 56

Oral erythromycin to eradicate organism and reduce spread Has not been shown to alter the course of the illness

Question 57

Cx of pertussis

Answer 57

Subconjunctival haemorrhage Pneumonia Bronchiectasis Seizures

Question 58

A mother presents to your GP surgery with her six month old daughter. She has been struggling to feed her daughter, and her health visitor found that she was small for her age. Her mother is exhausted as she says her daughter sleeps poorly. On examination, the baby is just below the 3rd centile in length. She has epicanthic folds and low set ears. Her neck appears short and she has micrognathia. You hear an ejection systolic murmur on auscultation. What is the most likely diagnosis? Fragile X syndrome Down's syndrome Patau syndrome Klinefelter's syndrome Turner's syndrome

Answer 58

Turner's syndrome is a genetic condition due to a loss or abnormality of one X chromosome. In infancy, children often have difficulty with feeding which contributes to poor weight gain, although the often have short stature too when older. Babies with Turner's syndrome often have multiple dysmorphic features, but a webbed neck is often classical. It is also associated with cardiac abnormalities, in this question aortic stenosis although others are also common. Chromosome analysis would be needed to confirm the diagnosis. While Down's syndrome babies would have many of the dysmorphic features, they would not usually have a webbed neck or micrognathia. They may have loose skin at the nape of the neck but not webbing. It is caused by Trisomy 21. Klinefelter's syndrome is caused by having an extra X chromosome. They are often tall in stature with small testes and gynaecomastia. They do not tend to have the dysmorphic features. Fragile X syndrome is due to a CGG repeat on the X chromosome. They tend to have learning difficulties, long ears, mitral valve prolapse and a large forehead and jaw. Patau's syndrome is caused by trisomy 13. They do tend to have intrauterine growth restriction leading to low birth weight, and can have congenital heart defects and ear abnormalities. However, they do not have webbing of the neck, and eye dysmorphic features tend to be microphthalmia or anophthalmia. They typically have rocker bottom feet and polydactyly.

Question 59

Features of Turner's syndrome

Answer 59

45XO Short stature Shield chest, widely spaced nipples Webbed neck Cardiac: bicuspid aortic valve, coarctation Primary amenorrhoea Cystic hygroma High-arched palate Short fourth metacarpal Multiple pigmented naevi Lymphoedema Increased incidence of autoimmune disease: thyroiditis and Crohn's especially

Question 60

A 2 year old boy presents to the GP with his mother. She is worried that he is not growing at the same rate as the other children at his play group. His mother describes foul smelling diarrhoea about 4-5 times a week, accompanied by abdominal pain. On examination he has a bloated abdomen and wasted buttocks. He has dropped 2 centile lines and now falls on the 10th centile. What is the most appropriate initial investigation? ## Footnote Stool sample IgA TTG antibodies Hydrogen breath test Endoscopy Abdominal xray

Answer 60

The most likely diagnosis here is coeliac disease, diagnosed using IgA TTG antibodies, as explained below. A stool sample would be diagnostic for gastroenteritis, in order to dictate which antibiotic should be used. The hydrogen breath test is used to diagnose irritable bowel syndrome or some food intolerances. Endoscopy is more commonly used in adults where cancer is suspected. An abdominal X-ray may be useful where obstruction is suspected. Coeliac disease is a digestive condition which is becoming increasingly common, and describes an adverse reaction to gluten. gluten is a protein found in wheat, barley and rye.

Question 61

Coeliac disease in children

Answer 61

Coeliac disease is caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption. Children normally present before the age of 3 years, following the introduction of cereals into the diet

Question 62

Which HLAs are associated with coeliac?

Answer 62

HLA-DQ2 (95%) HLA-B8 (80%)

Question 63

Features of coeliac in children?

Answer 63

May coincide with the introduction of cereals FTT Diarrhoea Abdominal distension Older children may present with anaemia May not be dxed until adulthood

Question 64

Answer 64

Villous atrophy- Coeliac Duodenal biopsy from a patient with coeliac disease. Complete atrophy of the villi with flat mucosa and marked crypt hyperplasia. Intraepithelial lymphocytosis. Dense mixed inflammatory infiltrate in the lamina propria.

Question 65

Answer 65

Turner syndrome 45XO

Question 66

Hirschprung's features

Answer 66

Hirschsprung's disease is caused by an aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses. Although rare (occurring in 1 in 5,000 births) it is an important differential diagnosis in childhood constipation Possible presentations neonatal period e.g. failure or delay to pass meconium older children: constipation, abdominal distension Associations 3 times more common in males Down's syndrome

Question 67

Klumpke's palsy

Answer 67

Klumpke's palsy occurs due to damage of the lower brachial plexus and commonly affects the nerves innervating the muscles of the hand.

Question 68

Erb's palsy

Answer 68

Erb's palsy occurs due to damage to the upper brachial plexus most commonly from shoulder dystocia. Damage to these nerve roots results in a characteristic pattern: adduction and internal rotation of the arm, with pronation of the forearm. This classic physical position is commonly called the 'waiter's tip'.

Question 69

A male child from a travelling community is diagnosed with measles. Which one of the following complications is he at risk from in the immediate aftermath of the initial infection? Arthritis Pancreatitis Infertility Subacute sclerosing panencephalitis Pneumonia

Answer 69

Subacute sclerosing panencephalitis is seen but develops 5-10 years following the illness. Pancreatitis and infertility may follow mumps infection Pneumonia

Question 70

Measles overview

Answer 70

Overview RNA paramyxovirus spread by droplets infective from prodrome until 4 days after rash starts incubation period = 10-14 days

Question 71

Measles clinical features

Answer 71

Prodrome: irritable, conjunctivitis, fever Koplik spots: grains of salt, on buccal mucosa Rash: starts behind ears, then to whole body, discrete maculopapular rash becoming blotchy and confluent

Question 72

Answer 72

Measles

Question 73

Answer 73

Koplik spots Pre-measles rash on buccal mucosa

Question 74

Cxs of measles

Answer 74

encephalitis: typically occurs 1-2 weeks following the onset of the illness) subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness febrile convulsions giant cell pneumonia keratoconjunctivitis, corneal ulceration diarrhoea increased incidence of appendicitis myocarditis

Question 75

Mx of measles contacts

Answer 75

if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced measles antibody develops more rapidly than that following natural infection) this should be given within 72 hours

Question 76

Def: nephrotic syndrome

Answer 76

Nephrotic syndrome is classically defined as a triad of proteinuria (\> 1 g/m^2 per 24 hours) hypoalbuminaemia (\< 25 g/l) oedema

Question 77

Nephrotic syndrome in children

Answer 77

In children the peak incidence is between 2 and 5 years of age. Around 80% of cases in children are due to a condition called minimal change glomerulonephritis. The condition generally carries a good prognosis with around 90% of cases responding to high-dose oral steroids. Other features include hyperlipidaemia, a hypercoagulable state (due to loss of antithrombin III) and a predisposition to infection (due to loss of immunoglobulins)

Question 78

Features of acute epiglottitis

Answer 78

Acute epiglottitis is rare but serious infection caused by Haemophilus influenzae type B. Prompt recognition and treatment is essential as airway obstruction may develop. Epiglottitis was generally considered a disease of childhood but in the UK it is now more common in adults due to the immunisation programme. The incidence of epiglottitis has decreased since the introduction of the Hib vaccine

Question 79

Clinical features of epiglottits

Answer 79

Features rapid onset high temperature, generally unwell stridor drooling of saliva

Question 80

Develpmental milestones social behaviour 6w

Answer 80

Smiles (Refer at 10w)

Question 81

Develpmental milestones social behaviour 3m

Answer 81

Laughs Enjoys friendly handling

Question 82

Develpmental milestones social behaviour 6m

Answer 82

Not shy

Question 83

Develpmental milestones social behaviour 9m

Answer 83

Shy

Question 84

Developmental milestones: feeding 6m

Answer 84

May put hand on bottle when being fed

Question 85

Developmental milestones: feeding 12-15m

Answer 85

Drinks from cup and uses spoon

Question 86

Developmental milestones: feeding 2y

Answer 86

Competent with sppon, doesn't spill cup

Question 87

Developmental milestones: feeding 3y

Answer 87

Uses spoon and fork

Question 88

Developmental milestones: dressing 12-15m

Answer 88

Helps getting dress/undressed

Question 89

Developmental milestones: dressing 18m

Answer 89

Takes of shoes, hat but unable to replace

Question 90

Developmental milestones: dressing 2y

Answer 90

Puts on hat and shoes

Question 91

Developmental milestones: dressing 4y

Answer 91

Can dress and undress independently except for laces and buttons

Question 92

Developmental milestones: play 9m

Answer 92

Peek a boo

Question 93

Developmental milestones: play 12m

Answer 93

Waves bye bye Plays pat a cake

Question 94

Developmental milestones: play 18m

Answer 94

Plays contentedly alone

Question 95

Developmental milestones: play 2y

Answer 95

Plays near others, not with them

Question 96

Developmental milestones: play 4y

Answer 96

Plays with other children

Question 97

A 7-year-old boy is brought in to the GP surgery with an exacerbation of asthma. On examination he has a bilateral expiratory wheeze but there are no signs of respiratory distress. His respiratory rate is 36 / min and PEF around 60% of normal. What is the most appropriate action with regards to steroid therapy? Oral prednisolone for 3 days Admit for intravenous steroids Give a stat dose of oral dexamethasone Double his usual beclometasone dose Do not give steroids

Answer 97

Oral prednisolone for 3 days

Question 98

2-5y/o Asthma Moderate attack

Answer 98

SpO2 \>92% No clinical features of severe asthma

Question 99

2-5y/o Asthma Severe attack

Answer 99

SpO2 \<92% Too breathless to talk or feed HR \>140 RR \>40 Use of accessory neck muscles

Question 100

2-5y/o Asthma Life-threatening attack

Answer 100

SpO2 \<92% Silent chest Poor respiratory effort Agitation Altered consciousness Cyanosis

Question 101

\>5y/o Asthma Moderate attack

Answer 101

SpO2 \>92% PEF \>50% predicted No clinical features of severe asthma

Question 102

\>5y/o Asthma Severe attack

Answer 102

SpO2 \<92 PEF 33-50 Can't complete sentences in one breath or too breathless to talk or feed HR \>125 RR \>30 Use of accessory neck muscles

Question 103

\>5y/o Asthma Life-threatening attack

Answer 103

SpO2 \<92 PEF \<33 Silent chest Poor respiratory effort Altered consciousness Cyanosis

Question 104

PEF in children

Answer 104

Attempt to measure in all children \>5

Question 105

Mx of mild-moderate acute asthma

Answer 105

Bronchodilator: Beta-2 agonist via a spacer (\<3y use a close-fitting mask) 1 puff every 15-30secs, up to a maximum of 10 puffs, repeat dose after 10-20 mins if necessary If symptoms are not controlled, repeat beta-2 and refer to hospital Steroid therapy: should be given to all children with asthma exacerbation Treatment for 3-5d

Question 106

Prednisolone dose 2-5y \>5y

Answer 106

Age Dose as per BTS Dose as per cBNF 2 - 5 years 20 mg o d1-2 mg/kg od (max 40mg) \> 5 years 30 - 40 mg od 1-2 mg/kg od (max 40mg)

Question 107

A 9-year-old boy is brought to surgery with recurrent headaches. What is the most common cause of headaches in children? Migraine Depression Refractive errors Tension-type headache Cluster headache

Answer 107

Migraine

Question 108

Features of Hand foot and mouth disease?:

Answer 108

Hand, foot and mouth disease is a self-limiting condition affecting children. It is caused by the intestinal viruses of the Picornaviridae family (most commonly coxsackie A16 and enterovirus 71). It is very contagious and typically occurs in outbreaks at nursery Clinical features mild systemic upset: sore throat, fever oral ulcers followed later by vesicles on the palms and soles of the feet

Question 109

Mx of hand foot and mouth?

Answer 109

Management general advice about hydration and analgesia reassurance no link to disease in cattle children do not need to be excluded from school\* \*The HPA recommends that children who are unwell should be kept off school until they feel better. They also advise that you contact them if you suspect that there may be a large outbreak.

Question 110

Answer 110

Hand foot and mouth disease

Question 111

Features of Croup?

Answer 111

Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases. Epidemiology peak incidence at 6 months - 3 years more common in autumn Features stridor barking cough (worse at night) fever coryzal symptoms

Question 112

Features of mild croup

Answer 112

Occasional barking cough No audible stridor at rest No or mild suprasternal and or intercostal recession Child is happy and is prepared to eat, drink and play

Question 113

Features of moderate croup

Answer 113

Frequent barking cough Easily audible stridor and rest Suprasternal and sternal wall retraction at rest No or little distress or agitation Child can be placated

Question 114

Features of severe croup

Answer 114

Frequent barking cough Prominent inspiratory and occasionally expiratory stridor at rest Marked sternal wall retractions Significant distress and agitation or lethargy or restlessness (sign of hypoxaemia) Tachycardia occurs with more sever obstructive symptoms and hypoxaemia

Question 115

Indications for admission in croup

Answer 115

Moderate or severe croup \<6m Known upper airway abnormality e.g. laryngomalacia, DS Uncertainty about ddx (acute epiglottitis, bacterial tracheitis, peritonsillar abscess, FBI)

Question 116

Mx of croup

Answer 116

Single dose of oral dexamethasone (0.15mg/kg) Emergency: High flow O2 Nebulised adrenaline

Question 117

The parents of a 14-month-old girl present to their GP. They have noticed that in some photos there is no 'red eye' on the left hand side. When you examine the girl you notice an esotropic strabismus and a loss of the red-reflex in the left eye. There is a family history of a grandparent having an enucleation as a child. What is the most likely diagnosis? Congenital hypertrophy of the retinal pigment epithelium Uveal malignant melanoma Neuroblastoma Retinoblastoma Congenital cataract

Answer 117

A congenital cataract may cause a loss of the red-reflex but is likely to have been detected at birth or during the routine baby-checks. It would also not explain the family history of enucleation. Retinoblastoma

Question 118

Features of retinoblastoma

Answer 118

Retinoblastoma is the most common ocular malignancy found in children. The average age of diagnosis is 18 months. Pathophysiology caused by a loss of function of the retinoblastoma tumour suppressor gene on chromosome 13 around 10% of cases are hereditary Possible features absence of red-reflex, repalced by a white pupil (leukocoria) - the most common presenting symptom strabismus visual problems

Question 119

Mx of retinoblastoma

Answer 119

Management enucleation is not the only option depending on how advanced the tumour is other options include external beam radiation therapy, chemotherapy and photocoagulation Prognosis excellent, with \> 90% surviving into adulthood

Question 120

Draw management of asthma in children under 5

Answer 120

Question 121

Draw management of asthma in children \>5

Answer 121

Question 122

What is the major risk factor for NRDS?

Answer 122

Prematurity

Question 123

What is the major risk factor for TTN?

Answer 123

C-sec

Question 124

What is the major risk factor for aspiration pneumonia?

Answer 124

Meconium staining

Question 125

What differentiates between NRDS and TTN?

Answer 125

Neonates with NRDS usually present with respiratory distress shortly after birth which usually worsens over the next few days. In contrast, TTN usually presents with tachypnoea shortly after birth and often fully resolves within the first day of life. A chest radiograph can be useful

Question 126

CXR in NRDS?

Answer 126

Diffuse ground glass lungs Low volumes Bell shaped throax

Question 127

Answer 127

NRDS

Question 128

CXR in TTN

Answer 128

Heart failure type pattern Intersitital oedema PLeural effusions But normal heart size in contrast to congenital heart disease

Question 129

Answer 129

TTN

Question 130

Features of Surfactant lung disease?

Answer 130

Surfactant deficient lung disease (SDLD, also known as respiratory distress syndrome and previously as hyaline membrane disease) is a condition seen in premature infants. It is caused by insufficient surfactant production and structural immaturity of the lungs The risk of SDLD decreases with gestation 50% of infants born at 26-28 weeks 25% of infants born at 30-31 weeks Other risk factors for SDLD include male sex diabetic mothers Caesarean section second born of premature twins Clinical features are those common to respiratory distress in the newborn, i.e. tachypnoea, intercostal recession, expiratory grunting and cyanosis Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border

Question 131

Mx of SDLD?

Answer 131

Management prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation oxygen assisted ventilation exogenous surfactant given via endotracheal tube

Question 132

How can primary, secondary and tertiary preventative measures be classified?

Answer 132

Preventive healthcare can be divided up into primary (preventing the accident/disease from happening), secondary (prevent injury from the accident/disease) and tertiary (limit the impact of the injury) prevention strategies

Question 133

Contraindications to IFV immunisation in children?

Answer 133

Contraindications immunocompromised aged \< 2 years current febrile illness or blocked nose/rhinorrhoea current wheeze (e.g. ongoing viral-induced wheeze/asthma) or history of severe asthma (BTS step 4) egg allergy pregnancy/breastfeeding if the child is taking aspirin (e.g. for Kawasaki disease) due to a risk of Reye's syndrome

Question 134

A mother is concerned about the risk of her son developing influenza. Her son is fit and otherwise well. Following NHS immunisation guidance, at what age should the child first be offered the influenza vaccine? 3 months 4 months 12-13 months 2-3 years 65 years

Answer 134

2-3y

Question 135

Suggestigve of constipation (\>=2) Stool pattern in Child \<1y

Answer 135

Fewer than 3 complete stools per week hard large stool Rabbit droppings

Question 136

Stool pattern suggestigve of constipation (\>=2) \>1y

Answer 136

Fewer than 3 complete stools per week Overflow soiling (commonly very loose, very smelly and passed without sensation) Rabbit droppings Large, infrequent stools that can block toilet

Question 137

Symptoms associated with defecation suggestigve of constipation (\>=2) \<1y

Answer 137

Distress on passing stool Bleeding associated with hard stool Straining

Question 138

Symptoms associated with defecation suggestigve of constipation (\>=2) \>1y

Answer 138

Poor appetite that improves with passage of large stool Waxing and waning of abdo pain with passage of stool Evidence of tenetive posturing Straining Anal pain

Question 139

Retentive posturing in examination?

Answer 139

Typical straight legged, tip toed, back arching

Question 140

Hx suggestigve of constipation (\>=2) \<1y

Answer 140

Previous episode of constipation Previous or current anal fissure

Question 141

Hx suggestigve of constipation (\>=2) \>1y

Answer 141

Previous epsiode Previous or currrent anal fissure Painful BM and bleeding associated with hard stools

Question 142

Causes of constipation

Answer 142

Idiopathic Dehydration Diet Medication e.g. opiates Anal fissure Over-enthusiastic potty training Hypothyroidism Hirschsprung's Hypercalcaemia LD

Question 143

What timing indicates idiopathic constipation?

Answer 143

Starts after a few weeks of life Obvious precipitating factors coinciding with the start of symptoms: fissure, change of diet, timing of potty/toilet training or acute events such as infections, moving house, starting nursery/school, fears and phobias, major change in family, taking medicines

Question 144

What is a red flag in constipation relating to timing?

Answer 144

Reported from birth or first few weeks of life

Question 145

Passage of meconium in idiopathic constipation?

Answer 145

\<48h

Question 146

Red flag in meconium passage?

Answer 146

\>48h

Question 147

Ribbon stools?

Answer 147

?Hirschprungs

Question 148

Faltering growth in constipation?

Answer 148

Amber flag

Question 149

Growth in idiopathic sontipation

Answer 149

Generally well Weight and height within normal limits, fit and active

Question 150

Red flag in constipation

Answer 150

Previously unkown or undiagnosed weakness in legs, locomotr delay

Question 151

Improtant determinant in idiopathic constipation?

Answer 151

Changes in infant formula Weaning Insufficient fluid intake or poor diet

Question 152

Abdominal distension in constipation?

Answer 152

Red flag symptom suggestive of underlying disorder

Question 153

What features suggest fecal impaction?

Answer 153

Symptoms of severe constipation Overflow soiling Faecal mass palpable in abdomen (DRE should only be performed by specialist)

Question 154

Mx of feacal impaction

Answer 154

Polyethylene glycoe 3350 + electroyles (using an escalating dose) is first line Stimulant laxative can be addied if first line does not lead to disimpaction after 2 weeks. Subsititue a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric plan is not tolerated. Inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain

Question 155

What type of laxative is Movicol Plain?

Answer 155

Osmotic

Question 156

Eg of a stimulant laxative?

Answer 156

Senna

Question 157

Maintenance therapy in constipation

Answer 157

very similar to the above regime, with obvious adjustments to the starting dose, i.e. first-line: Movicol Paediatric Plain add a stimulant laxative if no response substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if stools are hard continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce dose gradually

Question 158

What type of laxative is lactulose?

Answer 158

Osmotic

Question 159

Bulk forming laxatives?

Answer 159

ispaghula psyllium) husk, methylcellulose and sterculia

Question 160

Osmotic laxatives

Answer 160

lactulose, macrogols, phosphate enemas and sodium citrate enemas.

Question 161

Stimulant laxatives.

Answer 161

bisacodyl, docusate sodium, glycerol, senna andsodium picosulfate

Question 162

General points in Mx of constipation?

Answer 162

General points do not use dietary interventions alone as first-line treatment although ensure child is having adequate fluid and fibre intake consider regular toileting and non-punitive behavioural interventions for all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.

Question 163

Mx of infants not yet weaned with constipation

Answer 163

bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant's legs breast-fed infants: constipation is unusual and organic causes should be considered

Question 164

Mx of infants with constipation that have or are being weaned

Answer 164

Infants who have or are being weaned offer extra water, diluted fruit juice and fruits if not effective consider adding lactulose

Question 165

Exacerbations of chronic bronchitis

Answer 165

Amoxicillin or tetracycline or clarithromycin

Question 166

Uncomplicated community-acquired pneumonia

Answer 166

Amoxicillin (Doxycycline or clarithromycin in penicillin allergic, add flucloxacillin if staphylococci suspected e.g. In influenza)

Question 167

Pneumonia possibly caused by atypical pathogens

Answer 167

Clarithromycin

Question 168

Hospital-acquired pneumonia

Answer 168

Within 5 days of admission: co-amoxiclav or cefuroxime More than 5 days after admission: piperacillin with tazobactam OR a broad-spectrum cephalosporin (e.g. ceftazidime) OR a quinolone (e.g. ciprofloxacin)

Question 169

Lower urinary tract infection

Answer 169

Trimethoprim or nitrofurantoin. Alternative: amoxicillin or cephalosporin

Question 170

Acute pyelonephritis

Answer 170

Broad-spectrum cephalosporin or quinolone

Question 171

Acute prostatitis

Answer 171

Quinolone or trimethoprim

Question 172

Impetigo

Answer 172

Topical fusidic acid, oral flucloxacillin or erythromycin if widespread

Question 173

Cellulitis

Answer 173

Flucloxacillin (clarithromycin or clindomycin if penicillin-allergic)

Question 174

Erysipelas

Answer 174

Phenoxymethylpenicillin (erythromycin if penicillin-allergic)

Question 175

Animal or human bite

Answer 175

Co-amoxiclav (doxycycline + metronidazole if penicillin-allergic)

Question 176

Mastitis during breast-feeding

Answer 176

Flucloxacillin

Question 177

Throat infections

Answer 177

Phenoxymethylpenicillin (erythromycin alone if penicillin-allergic)

Question 178

Sinusitis

Answer 178

Amoxicillin or doxycycline or erythromycin

Question 179

Otitis media

Answer 179

Amoxicillin (erythromycin if penicillin-allergic)

Question 180

Otitis externa\*

Answer 180

Flucloxacillin (erythromycin if penicillin-allergic) \*a combined topical antibiotic and corticosteroid is generally used for mild/moderate cases of otitis externa

Question 181

Gonorrhoea

Answer 181

Intramuscular ceftriaxone + oral azithromycin

Question 182

Chlamydia

Answer 182

Doxycycline or azithromycin

Question 183

Pelvic inflammatory disease

Answer 183

Oral ofloxacin + oral metronidazole or intramuscular ceftriaxone + oral doxycycline + oral metronidazole

Question 184

Syphilis

Answer 184

Benzathine benzylpenicillin or doxycycline or erythromycin

Question 185

Bacterial vaginosis

Answer 185

Oral or topical metronidazole or topical clindamycin

Question 186

Clostridium difficile

Answer 186

First episode: metronidazole Second or subsequent episode of infection: vancomycin

Question 187

Campylobacter enteritis

Answer 187

Clarithromycin

Question 188

Salmonella (non-typhoid)

Answer 188

Ciprofloxacin

Question 189

Shigellosis

Answer 189

Ciprofloxacin

Question 190

Meningitis Neonatal to 3 months

Answer 190

Group B Streptococcus: usually acquired from the mother at birth. More common in low birth weight babies and following prolonged rupture of the membranes E. coli and other Gram -ve organisms Listeria monocytogenes

Question 191

Meningitis 1 month to 6 years

Answer 191

Neisseria meningitidis (meningococcus) Streptococcus pneumoniae (pneumococcus) Haemophilus influenzae

Question 192

Meningitis \>6y

Answer 192

Neisseria meningitidis (meningococcus) Streptococcus pneumoniae (pneumococcus)

Question 193

Throat examination in Croup?

Answer 193

Should be avoided as it may precipitate airway obstruction

Question 194

A newborn baby is transferred to the neonatal intensive care unit shortly after birth due to respiratory distress. An x-ray taken on arrival is shown below: What is the diagnosis? Bronchopulmonary dysplasia Respiratory distress syndrome Left-sided neonatal bronchiectasis Congenital diaphragmatic hernia Left pneumothorax

Answer 194

Bowel loops can be seen in the left side of the thoracic cavity.

Question 195

Features of congenital diaphragmatic hernia

Answer 195

Occurs in 1:2000 Characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm Can result in pulmonary hypoplasia and HTN which causes RDS shortly after birth

Question 196

Px for congenital diaphramatic hernia

Answer 196

50% survive despite intervention

Question 197

What is the most common type of congenital diaphramatic hernia?

Answer 197

Bochdalek hernia 85% cases Left sided, posterolateral

Question 198

Features of: Chickenpox

Answer 198

Fever initially Rash starting on head/trunk before spreading Initially macular, then papular, then vessciular Normally mild systemic upset

Question 199

Answer 199

Chicken pox

Question 200

Features of measles

Answer 200

Prodrome: irritable, conjuncitivits, fever Koplik spots Rash starting behind ears, spreading to the whole body Initially discrete maculopapular rash that becomes blotchy and confluent

Question 201

Features of mumps

Answer 201

Fever, malaise, muscular pain Parotitis initially unilateral becoming bilateral in 70%

Question 202

Features of Rubella

Answer 202

Pink maculopapular rash initially on face before spreading to the whole body, usually faind by the 3-5th day Suboccipital and postauricular lymphadenopathy

Question 203

Answer 203

Rubella

Question 204

Features of erythema infectiosum

Answer 204

AKA slapped cheek syndrome Caused by parvovirus B19 Lethargy, fever, headache Slapped cheek rash spreading to proximal arms and extensor surfaces

Question 205

Answer 205

Erythema infectiosum

Question 206

Features of Scarlet fever

Answer 206

Reaction to erythrogenci toxins produced by group A haemolytic strep Fever, malaise, tonsilitis Strawberry tongue Fine punctate erythema sparing face

Question 207

Answer 207

Scarlet Fever

Question 208

Features of hand, foot and mouth disease

Answer 208

Caused by coxsackie A16 virus Mild systemic upset: sore throat, fever Vesciles in the mouth and on the palms and soles of the feet

Question 209

Answer 209

Hand foot and mouth

Question 210

Scarlet fever features

Answer 210

Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes). It is more common in children aged 2 - 6 years with the peak incidence being at 4 years. Scarlet fever has an incubation period of 2-4 days and typically presents with: fever malaise tonsillitis 'strawberry' tongue rash - fine punctate erythema ('pinhead') which generally appears first on the torso and spares the face although children often have a flushed appearance with perioral pallor. The rash often has a rough 'sandpaper' texture. Desquamination occurs later in the course of the illness, particularly around the fingers and toes

Question 211

Dx of scarlet fever

Answer 211

Throat swab usually taken but antibiotic treatment should be commenced immediately

Question 212

Mx of scarlet fever

Answer 212

Oral penicillin V (penallergic: azithromycin) Children can return to school 24h after commencing antibiotics Notifiable disease

Question 213

Cx of Scarlet fever?

Answer 213

Otitis media: most common Rheumatic fever: typically 20d after infection Acute GN

Question 214

Autosomal recessive conditions

Answer 214

Autosomal recessive conditions are often thought to be 'metabolic' as opposed to autosomal dominant conditions being 'structural', notable exceptions: some 'metabolic' conditions such as Hunter's and G6PD are X-linked recessive whilst others such as hyperlipidemia type II and hypokalemic periodic paralysis are autosomal dominant some 'structural' conditions such as ataxia telangiectasia and Friedreich's ataxia are autosomal recessive

Question 215

Inheritance: Albinism

Answer 215

AR

Question 216

Inheriance: Congenital adrenal hyperplasia

Answer 216

AR

Question 217

Inheritance: Ataxia telangiectasia

Answer 217

AR

Question 218

Inehritance: Familial Mediterranean fever

Answer 218

AR

Question 219

Inheritance: Fanconi anaemia

Answer 219

AR

Question 220

Inheritance: Friedreichs ataxia

Answer 220

AR

Question 221

Inheritance: Gilber'ts

Answer 221

AR (although some textbooks will still say AD)

Question 222

Glycogen storage disease inheritance

Answer 222

AR

Question 223

Hamochromatosis inheritance

Answer 223

AR

Question 224

Homocystinuria inheritance

Answer 224

AR

Question 225

Lipid storage disease: Tay-Sach's, Gaucher, Niemann-Pick Inheritance

Answer 225

AR

Question 226

Mucopolysaccharidoses: Hurler's Inheritance

Answer 226

AR

Question 227

PKU inheritance

Answer 227

AR

Question 228

Sickle cell inheritance

Answer 228

AR

Question 229

Thalassaemia inheritance

Answer 229

AR

Question 230

Wilson's inheritance

Answer 230

AR

Question 231

Characteristic symptoms in ADHD?

Answer 231

Extreme restlessness Poor concentration Uncontrolled activity Impusliveness

Question 232

Answer 232

Question 233

Patau syndrome (trisomy 13)

Answer 233

Microcephalic, small eyes Cleft lip/palate Polydactyly Scalp lesions

Question 234

Edward's syndrome (trisomy 18)

Answer 234

Micrognathia Low-set ears Rocker bottom feet Overlapping of fingers

Question 235

Fragile X

Answer 235

Learning difficulties Macrocephaly Long face Large ears Macro-orchidism

Question 236

Noonan syndrome

Answer 236

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Question 237

Pierre-Robin syndrome\*

Answer 237

\*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome is autosomal dominant so there is usually a family history of similar problems Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Question 238

Prader-Willi syndrome

Answer 238

Hypotonia Hypogonadism Obesity

Question 239

William's syndrome

Answer 239

Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis

Question 240

Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis

Answer 240

William's syndrome

Question 241

Hypotonia Hypogonadism Obesity

Answer 241

Prader-Willi syndrome

Question 242

Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Answer 242

Pierre-Robin syndrome\*

Question 243

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Answer 243

Noonan syndrome

Question 244

Learning difficulties Macrocephaly Long face Large ears Macro-orchidism

Answer 244

Fragile X

Question 245

Micrognathia Low-set ears Rocker bottom feet Overlapping of fingers

Answer 245

Edward's syndrome (trisomy 18)

Question 246

Microcephalic, small eyes Cleft lip/palate Polydactyly Scalp lesions

Answer 246

Patau syndrome (trisomy 13)

Question 247

What is the difference between primary, secondary and tertiary prevention strategies?

Answer 247

Preventive healthcare can be divided up into primary (preventing the accident/disease from happening), secondary (prevent injury from the accident/disease) and tertiary (limit the impact of the injury) prevention strategies

Question 248

What is the most common cause of childhood death in 1-15y/o?

Answer 248

Accidents

Question 249

What Ixs should be performed in infants \<3m old with fever?

Answer 249

FBC Blood culture CRP Urine dip CXR if respiratory signs are present Stool culture if diarrhoea is present

Question 250

What causes head lice?

Answer 250

Pediculosis capitis

Question 251

What is the dx of head live?

Answer 251

Treatment only if living lice found Malathion, wet combing, dimeticone, isopropyl myristate and cyclomethicone. School exclusion is not advised

Question 252

HAP Rx

Answer 252

Within 5d of admission: co amoxiclav or cefuroxime \>5d: piperacilline with tazobactam OR a broad-spectrum cephalosporin OR a quinolone (provides pseudomonas cover)

Question 253

Risk of DS by maternal age?

Answer 253

One way of remembering this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for every extra 5 years of age

Question 254

What 3 features must be present to dx autism?

Answer 254

All 3 of the following features must be present for a diagnosis to be made global impairment of language and communication impairment of social relationships ritualistic and compulsive phenomena

Question 255

What syndromes are assocaited with autism?

Answer 255

Fragile X Rett's

Question 256

What are hte criteria for admission in bronchiolitis?

Answer 256

Apnoea (observed or reported) Persistent oxygen saturation of \<92% in air Inadequate oral fluid intake (\<50% of normal fluid intake) Persisting severe respiratory distress, for example grunting, marked chest recession, or a respiratory rate of over 70 breaths/minute.

Question 257

Ix of bronchiolitis?

Answer 257

Can be done using fluorescent Ab test on nasopharyngeal secretions

Question 258

Mx of RSV

Answer 258

Admit if fulfils criteria Deliver humidifed oxygen best through head box. Level can be determiend with pulse oximetry. Fluids and feed may need to be given by NG tube or IV. Only 5% require venitlation.

Question 259

What is the most common cause of food poisoning in the UK?

Answer 259

Campylobacter. \<5y/o and \>60 y/o are at greater risk

Question 260

Features of campylobacter

Answer 260

Campylobacter is the commonest bacterial cause of infectious intestinal disease in the UK. The majority of cases are caused by the Gram-negative bacillus Campylobacter jejuni. It is spread by the faecal-oral route and has an incubation period of 1-6 days. Features prodrome: headache malaise diarrhoea: often bloody abdominal pain

Question 261

Mx of Campylobacter

Answer 261

Usually self-limiting BNF advises antibiotics if immunocompromised. CKS also advise antibiotics if severe symptoms (high fever, bloody diarrhoea, \>8 stools/d). First line antibiotic is clarithromycin

Question 262

Cxs of campylobacter infection

Answer 262

GB syndrome Reiter's syndrome Septicaemia, endocarditis, arthritis

Question 263

A 2-year-old girl is brought to her GP because her mother has noticed she is constantly itching her bottom at night. Her mother says she has noticed some strange looking white bits when she wipes her daughters bottom following a bowel motion. What is the most appropriate management option? ## Footnote Prescribe 14 days of daily miconazole for whole household and issue hygiene advice. Issue hygiene advice only. Prescribe a single dose of mebendazole for the daughter and issue hygiene advice. Prescribe a single dose of mebendazole for the whole household and issue hygiene advice. Prescribe topical clotrimazole for 2 weeks and issue hygiene advice.

Answer 263

This child is highly likely to have a threadworm infection with symptoms of perianal itching that is worse at night. It is also possible to see threadworms, described as small threads of slowly-moving white cotton either around the anus or in the stools. The risk of transmission in families is as high as 75%, and asymptomatic infestation is common. For this reason an anthelmintic drug (mebendazole) should be given as a single dose to all household members.

Question 264

Features of threadworm infection

Answer 264

Asymptomactic in 90% Perianal itching, particulrly at night. Girls may have vulval symptoms. Dx can be made by applyoing sellotape to the perianal area and sending it to the laboratory for microscopy to identify the eggs.

Question 265

First line antihelmintic for children \>6m

Answer 265

mebendazole

Question 266

Paediatric PLS

Answer 266

Unresponsive Help Open airway Look, listen, feel for breathing 5 rescue breaths Circulation? 15 chest compressions:2

Question 267

What are hte contraindications to the MMR?

Answer 267

severe immunosuppression allergy to neomycin children who have received another live vaccine by injection within 4 weeks pregnancy should be avoided for at least 1 month following vaccination immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are present)

Question 268

Adverse affects of the MMR

Answer 268

malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days

Question 269

A mother comes to surgery with her 6-year-old son. During the MMR scare she decided not to have her son immunised. However, due to a recent measles outbreak she asks if he can still receive the MMR vaccine. What is the most appropriate action? Arrange for measles immunoglobulin to be given Cannot vaccinate at this age as live vaccine Give separate measles vaccine Give MMR with repeat dose in 3 months Give MMR with repeat dose in 5 years

Answer 269

The Green Book recommends allowing 3 months between doses to maximise the response rate. A period of 1 month is considered adequate if the child is greater than 10 years of age. In an urgent situation (e.g. an outbreak at the child's school) then a shorter period of 1 month can be used in younger children.

Question 270

Transient synovitis

Answer 270

Acute onset Usually accompanies viral infections, but the child is well or has a mild fever More common in boys, aged 2-12 years

Question 271

Septic arthritis/osteomyelitis

Answer 271

Unwell child, high fever

Question 272

Juvenile idiopathic arthritis

Answer 272

Limp may be painless

Question 273

Trauma

Answer 273

History is usually diagnostic

Question 274

Development dysplasia of the hip

Answer 274

Usually detected in neonates 6 times more common in girls

Question 275

Perthes disease

Answer 275

More common at 4-8 years Due to avascular necrosis of the femoral head

Question 276

Slipped upper femoral epiphysis

Answer 276

10-15 years - Displacement of the femoral head epiphysis postero-inferiorly

Question 277

Criteria for admission in sickle cell crisis?

Answer 277

Admit all people with clinical features of a sickle cell crisis to hospital unless they are: A well adult who only has mild or moderate pain and has a temperature of 38°C or less. A well child who only has mild or moderate pain and does not have an increased temperature. This is based on the recommendation that a fever with no identified source associated with a sickle cell crisis needs bloods and cultures taken to look for the possible source of infection and early treatment as there is a higher risk of severe infections due to hyposplenism. Consider admission if the person presents with a fever but is otherwise generally well. Admission is not necessarily required if the source of infection is obvious (such as a viral illness) and can be managed in the community. Have a low threshold for admission: In a child. If the person has a temperature over 38°C (as there is a risk of rapid deterioration). If the person has chest symptoms (as acute chest syndrome may develop quickly). Make sure that the person with chest symptoms and their family understand the importance of seeking urgent medical advice if their clinical state deteriorates, especially if breathing becomes faster or more laboured. Whenever possible, admit the person to the specialist centre that has their records.

Question 278

Management of sickle cell crises

Answer 278

Analgesia: opiates Rehydrate O2 Consider antibiotics Blood transfusion Exchange transfusion e.g. if neurological complications

Question 279

How can hypotonia be classified?

Answer 279

May be central or related to nerve and muscle problems. Acutely ill child e.g. septicaemia may also be hypotonic on examination. Hypotonia associated with encephalopathy in the newborn period is most likely caused by hypoxic ischaemic encephalopathy

Question 280

Central causes of hypotonia?

Answer 280

DS Prader Willi Hypothyroid Cerebal palsy- hypotonia may preced the development of spasticity

Question 281

Neurological and muscular causes of hypotonia

Answer 281

Spinal muscular atrophy Spina bifida GB syndrome MG Muscular dystrophy Myotonic dystrophy

Question 282

myotonic dystrophy

Answer 282

Myotonic dystrophy (dystrophia myotonica, myotonia atrophica) is a chronic, slowly progressing, highly variable, inherited multisystemicdisease. It is an autosomal-dominant disease.It is characterized by wasting of the muscles (muscular dystrophy), cataracts, heart conduction defects, endocrine changes, and myotonia.[1] There are two main types of myotonic dystrophy. Myotonic dystrophy type 1 (DM1), also called Steinert disease, has a severe congenital form and an adult-onset form. Myotonic dystrophy type 2 (DM2), also called proximal myotonic myopathy (PROMM) is rarer than DM1 and generally manifests with milder signs and symptoms. Myotonic dystrophy can occur in people of any age. Both forms of the disease display an autosomal-dominant pattern of inheritance. Both "DM1" and "DM2" have adult-onset forms.

Question 283

Which one of the following statements regarding scabies is false? All members of the household should be treated Typically affects the fingers, interdigital webs and flexor aspects of the wrist in adults Scabies causes a delayed type IV hypersensitivity reaction Patients who complain of pruritus 4 weeks following treatment should be retreated Malathion is suitable for the eradication of scabies

Answer 283

It is normal for pruritus to persist for up to 4-6 weeks post eradication

Question 284

Features opf Scabies

Answer 284

Scabies is caused by the mite Sarcoptes scabiei and is spread by prolonged skin contact. It typically affects children and young adults. The scabies mite burrows into the skin, laying its eggs in the stratum corneum. The intense pruritus associated with scabies is due to a delayed type IV hypersensitivity reaction to mites/eggs which occurs about 30 days after the initial infection.

Question 285

Clinical features of scabies

Answer 285

Widespread prutirus Linear burrows on the side of fingers, interdigital webs and flexor aspects of hte wrist. Infants the face and scalp may be affected. 2o features are due to scratching excoriation, infection

Question 286

Mx of scabies

Answer 286

Permethrin 5% is first line Malathion is second line. Guidance on use. Pruritus persists 4-6w post eradication

Question 287

When is crusted scabies seen? Mx

Answer 287

Crusted scabies is seen in patients with suppressed immunity, especially HIV. The crusted skin will be teeming with hundreds of thousands of organisms. Ivermectin is the treatment of choice and isolation is essentia

Question 288

Answer 288

Crusted (Norweigan) scabies

Question 289

Risk factors for DDH

Answer 289

Female sex x6 Breech presentation Positive Fhx First born children Oligohydramnios Birthweight \>5kg Congenital calacenovalgus foot deformity

Question 290

Barlow test

Answer 290

Barlow maneuver. (A) The leg is pulled forward and then (B) adducted in an attempt to dislocate the femur. The Barlow test is a provocative maneuver used to diagnose a dislocatable hip. With the infant in a supine position, the hips are flexed to 90° and abducted. The thigh is grasped, and the leg is gently adducted while applying downward and lateral pressure (Fig 8A and B). A palpable clunk or movement indicates that the femoral head dislocates by sliding over the posterior rim of the acetabulum

Question 291

Ortolani test

Answer 291

Ortolani maneuver. (A) Initial downward pressure further dislocates the hip, which then (B) relocates as the thigh is adducted. A palpable “clunk” will be noted The Ortolani maneuver moves a dislocated hip back into the socket, creating a distinct, palpable sensation. To perform the Ortolani maneuver, place your index and middle fingers along the greater trochanter of the femur and your thumb along the inner thigh (Fig 7A and B). With the infant's legs in a neutral position, flex the infant's hips 90°. Gently abduct the hips while lifting forward on the femur. A positive Ortolani sign is noted if the hip is dislocated, by a characteristic clunk that is felt as the femoral head slides over the posterior rim of the acetabulum and is reduced

Question 292

Confirmation of dx of DDH

Answer 292

USS

Question 293

Mx of DDH

Answer 293

Most unstable hips will spontaneously stabilise by 3-6w Pavlik harnesses in children younger than 3-5m Older children may require sx

Question 294

A 3-year-old girl presents with a 3 day history of fever and bloody diarrhoea. Over the past 24 hours she has had 5 episodes of loose bloody stools. On examination she has a temperature of 39.6ºC, a heart rate of 175 bpm and her abdomen is soft with generalised tenderness. It is also noted that she has a reduced urinary output. Blood tests show a haemolytic anaemia and raised urea. What is the most likely diagnosis? Campylobacter gastroenteritis Salmonella gastroenteritis Norovirus Rotavirus Escherichia coli gastroenteritis

Answer 294

A short history of bloody diarrhoea is very suggestive of haemorrhagic gastroenteritis which can occur due to a variety of pathogens including Campylobacter, Salmonella and Escherichia coli. In this case, the haemolytic anaemia and raised urea suggest haemolytic uraemic syndrome. Haemolytic anaemia and renal failure form two parts of the classic triad of haemolytic uraemic syndrome. The third part of the triad is thrombocytopenia. It is usually caused by Escherichia coli subtype 0157. Treatment is supportive as antibiotics are contraindicated.

Question 295

How can E Coli be classified?

Answer 295

`According to its antigens which may trigger an immune response O: LPS K: Capsule H: Flagellin

Question 296

What E Coli serotype usually causes Neonatal meningitis?

Answer 296

K-1 (capsular antigen)

Question 297

A 9 year old boy is brought to the emergency department by ambulance. For approximately 24 hours he has had nausea and vomiting. However, he has now developed acute abdominal pain and when he arrives in the emergency department his breathing is laboured, deep and of a gasping nature. He is usually fit and well and is not prescribed any medication. Blood results show the following: Na+130 mmol/l K+3.5 mmol/l HCO3-19 mmol/l What is the likely cause? Sepsis Rotavirus Intestinal obstruction Meningitis Diabetic ketoacidosis

Answer 297

The patient in this scenario has developed diabetic ketoacidosis (DKA). The important pieces of information to consider when answering this question are his acute presentation and the blood results. This patient has presented to the emergency department with nausea, vomiting and acute abdominal pain. These are all symptoms of diabetic ketoacidosis. Furthermore, the laboured, deep breathing that is mentioned is Kussmaul's breathing, which is witnessed in DKA and metabolic acidosis. Kussmaul's breathing occurs whereby excess CO2 is exhaled as a compensatory mechanism for an increased blood pH. The recognition of Kussmauls breathing in this question is one of the major factors in getting this question correct, as you would not expect to see this phenomenon in the other 4 possible answers. The blood results are concurrent with a diagnosis of DKA. Bloods will often show a hyponatraemia, low bicarbonate and a hypokalaemia in severe cases. The low bicarbonate in this question gives the indication that there is an acidosis in this patient, which helps in deriving the correct answer. Taking into account all other answers, they each could explain some of the symptoms of this child. However, the low bicarbonate, his symptoms and Kussmaul's respirations should lead to a working diagnosis of diabetic ketoacidosis in this patient.

Question 298

Kussmaul's breathing

Answer 298

laboured, deep breathing that is mentioned is Kussmaul's breathing, which is witnessed in DKA and metabolic acidosis. Kussmaul's breathing occurs whereby excess CO2 is exhaled as a compensatory mechanism for an increased blood pH

Question 299

What are the most common precipitating factors for DKA?

Answer 299

Infection Missed insulin MI

Question 300

Features of DKA

Answer 300

Abdo pain Polyuria, polydipsia, dehydration Kussmaul respiration Acetone smelling breath

Question 301

What are the causes of death in DKA? Other complications?

Answer 301

Cerebral oedema Hypokalaemia Aspiration pneumonia Hypoglycaemia Hypokalaemia Systemic infections Appendicitis Pulmonary oedema Hyperosmolar hyperglycaemia non-ketotic coma

Question 302

What are the criteria for DKA

Answer 302

Glucose \>11 or known DM pH \<7.3 Bicarbonate \<15mmol Ketones \>3mmol or ++ on dipstick

Question 303

Mx of DKA

Answer 303

ABCD Correct dehydration Replace insulin Replace potassium Phosphate replacement Anticoagulation Once KA has resolved: Continue IV fluids until patient is drinking and tolerating food Change to subcut insuline once blood ketones \<1mmol Monitor until biochemistry has normalised

Question 304

Correcting dehydration in DKA

Answer 304

Assess dehydration: ideally weigh patient. Patients with \<5% dehydration who are not clinically unwell can be given oral rehydration with subcut insulin. If patient is severely dehydrated or schokled: 10ml/kg 0.9% saline as a bolus up to 30ml/kg Calculate deficit and replace over 48h along with usual maintenance requirements using 500ml of 0.9% saline, initially contianing 20mmol KCl, which can be changed to 0.45% saline and 20mmol KCl once BG has fallen to 12-15 mmol NB neonates may require larger volumes Fluid replacement should be monitored as some patients may experience massive diuresis

Question 305

Fluids in DKA

Answer 305

10ml/kg bolus 0.9% salin up to 30ml Calculate deficit + maintenance Over first 48h: 0.9% 500ml saline + 20mmol KCl until BG \<15 Then 0.45% saline + 20mmol KCl

Question 306

What can be used to clinically assess dehydration

Answer 306

CRT Skin turgor Respiratory pattern Dry mucous membranes Sunken eyes Weak pulses Cool peripheries Hypotension and oliguria which are late signs in children

Question 307

Mild dehydration

Answer 307

3%: only just clinically detectable

Question 308

Moderate dehydration

Answer 308

5%: dry mucous membranes and reduced skin turgor

Question 309

Severe dehydration

Answer 309

8% As for 5% but with sunken eyes and prolonged CRT

Question 310

Shocked in dehydration

Answer 310

Severely ill, with poor perfusion and thready rapid pulse Hypotension is a late sign and is not always present

Question 311

Replacing insulin in DKA

Answer 311

IV fluids and K replacement should occur 1-2h before starting. Early insulin associated with increased chacnce of developing cerebral oedema IV infusion at 0.1U/kg/h (can be 0.05U in younger children) Aim is to reduce BG by \<4mmol/h. If the BG drops below 8mmol add a sideline of 10% glucose and titrate to 8-12mmol but do not decrease the insulin infusion.

Question 312

Replacing K in DKA

Answer 312

Always depletion in total body potassium however initial serum values may not necessarily be low. K replacement should be started if patient is hypokalaemic, if not it should be when insulin is started. If the patient is hyperkalaemic, do not start replacement until U/O documented

Question 313

Phosphate replacement in DKA

Answer 313

Phosphate loss can be aggravated by insulin therapy. If associated with neurology, hypophosphataemia can be treated using K phosphate salts as an alternative to KCl

Question 314

Anticoagulation in DKA

Answer 314

Femoral line insertion associated with femoral vein thrombosis and these patients must be anticoagulated. May also be indicated in patients who are significantly hyperosmolar

Question 315

Monitoring of DKA

Answer 315

Obs Fluid blaance ECG CBG hourly Capillary blood ketones 1-2hourly Twice-daily weights Bloods every 2-4hrs Neurological investigations looking for indications of cerebral oedema.

Question 316

Mx of suspected cerebral oedema

Answer 316

Mannitol 0.1-1g/kg IV immediately over 20 minutes.

Question 317

Symptoms of cerebral oedema

Answer 317

Headache Vomiting Confusion or irritibaility Rising BP and bradycardia Decreased O2 saturation Focal neurology Papilloedema (late sign)

Question 318

Risk factors for cerebral oedema

Answer 318

Younger age New-onset DM Longer duration of symptoms Use of bicarbonate in management of KA

Question 319

Treatment of cerebral oedema

Answer 319

Exclude hypoglycaemia Mannitol Reduce rate of fluid admin Elevate head of the bed Transfer to ICU

Question 320

Cause of roseola infantum

Answer 320

AKA exanthem subitum Common disease of infancy caused by HHV6. Incubation period of 5-15d and affects children of 6m-2y

Question 321

Features of roseola infantum

Answer 321

high fever: lasting a few days, followed by a maculopapular rash febrile convulsions occur in around 10-15% diarrhoea and cough are also commonly seen

Question 322

Potential complications of HHV6 infection

Answer 322

aseptic meningitis hepatitis

Question 323

Answer 323

Roseola infantum (HHV6)

Question 324

Def: squint

Answer 324

Squint (strabismus) is characterised by misalignment of the visual axes. Squints may be divided into concomitant (common) and paralytic (rare)

Question 325

Cause of concomitant squint

Answer 325

Due to imablance in extraocular muscles Convergent is more common than divergent

Question 326

Cause of paralytic squint

Answer 326

Due to paralysis of extraocular muscles

Question 327

What is the corneal light reflection test

Answer 327

Used to identify squint Hold light source 30cm from the child's face

Question 328

Mx of strabismus

Answer 328

Eye patches may prevent amblyopia Referral to secondary care

Question 329

Ambylopia

Answer 329

Amblyopia, also known as lazy eye, is a vision development disorder in which an eye fails to achieve normal visual acuity, even with prescription eyeglasses or contact lenses. Amblyopia begins during infancy and early childhood. In most cases, only one eye is affected.

Question 330

What can be used to identify the nature of the squint

Answer 330

The cover test

Question 331

Presentation of UTI in childhood

Answer 331

Infants: poor feeding, vomiting, irritability Younger children: abdominal pain, fever, dysuria Older: dysuria, frequency, haematuria Features suggestive of upper UTI: T \>38, loin pain/tenderness

Question 332

Indications for checking urine in a nchild

Answer 332

Any symptoms suggestive Unexplained fever of 38 Alternative site of infection who remain unwell

Question 333

Collection of urine

Answer 333

Clean catch is preferrable If not possible then urine colleciton pads should be used. Suprapubic aspiration can be used if non-invasive methods are not possible

Question 334

Mx of UTI

Answer 334

\<3m: immediate referrl to paediatrirican \>3m with upper UT: consider admission, oral antibiotics including cephalosporin or co-amoxiclav should be given for 7-10m Lower UTI: oral antibiotics for 3d according to local guidelines (trimethoprim, nitrofurantoin, cepahlosporin, amoxicillin) Antibiotic prophylaxis not given after the first UTI but should be considered with recurrent UTIs

Question 335

Features of seborrhoeic dermatitis

Answer 335

Seborrhoeic dermatitis is a relatively common skin disorder seen in children. It typically affects the scalp ('Cradle cap'), nappy area, face and limb flexures. Cradle cap is an early sign which may develop in the first few weeks of life. It is characterised by an erythematous rash with coarse yellow scales.

Question 336

Mx of seborrhoeic dermatitis

Answer 336

Mild-moderate: baby shampoo and baby oils Severe: mild topical steroids e.g. 1% hydrocortisone Tends to resolve by 8m

Question 337

Answer 337

Seborrhoeic dermatitis (cradle cap)

Question 338

Def: anapylaxis

Answer 338

Anaphylaxis may be defined as a severe, life-threatening, generalised or systemic hypersensitivity reaction.

Question 339

Causes of anaphylaxis in children

Answer 339

Common identified causes of anaphylaxis food (e.g. Nuts) - the most common cause in children drugs venom (e.g. Wasp sting)

Question 340

Dose in anaphylaxis \<6m

Answer 340

150microg adrenaline 25mg hydrocortisone 250ug/kg chlorphenamine

Question 341

Dose in anaphylaxis 6m-6y

Answer 341

150microg adrenaline 50mg hydrocortisone 2.5mg chlorphenamine

Question 342

Dose in anaphylaxis 6-12y

Answer 342

300 microg adrenaline 100mg hydrocortisone 5mg chlorphenamine

Question 343

Dose in anaphylaxis Adult and child \>12

Answer 343

500ug adrenaline 200mg hydrocortisone 10mg chlorphenamine

Question 344

Adrenaline in anaphylaxis

Answer 344

Can be repeated every 5 minutes if necessary

Question 345

What is the best site for IM injection in children of adrenaline

Answer 345

Anterolateral aspect of the middle third of the thigh

Question 346

Def: CMPI/CMPA

Answer 346

Cow's milk protein intolerance/allergy (CMPI/CMPA) occurs in around 3-6% of all children and typically presents in the first 3 months of life in formula fed infants, although rarely it is seen in exclusively breastfed infants. Both immediate (IgE mediated) and delayed (non-IgE mediated) reactions are seen. The term CMPA is usually used for immediate reactions and CMPI for mild-moderate delayed reactions.

Question 347

Dx of cows milk protein intolerance/allergy

Answer 347

Often clinical (elimination of cow's milk protein) Skin prick/patch testing Total IgE and specific IgE (RAST) for cow's milk protein

Question 348

Mx of cow's milk protein intolerance/allergy

Answer 348

If FTT-\> paediatirician If formula fed: Extensive hydrolysed formula milk is first line replacement for infants with mild-moderate symptoms Amino-acid based formula in infants with severe CMPA or if no response to eHF 10% also intolerant to soy milk If breast fed: Continue breastfeeding Eliminate cow's milk protein from maternal diet Use eHF milk when breast feeding stops until 12m of age and at least 6m Usually resolves by 1-2y/o Challenge often performed in hospital due to risk of anaphylaxis

Question 349

A baby boy born 6 hours ago has an APGAR score of 10. He is not cyanosed, has a pulse of 140, cries on stimulation, his arms and legs resist extension and he has a good cry, He appears jaundiced. What is the most appropriate action? Encourage the mother to sit with the baby in sunlight Arrange a blood transfusion Start phototherapy Prescribe intravenous immunoglobulin Measure and record the serum bilirubin level urgently.

Answer 349

Measure and record the serum bilirubin level urgently (within 2 hours) in all babies with suspected or obvious jaundice in the first 24 hours of life since this is likely to be pathological rather than physiological jaundice. NICE CG98

Question 350

Jaundice \<24h y/o

Answer 350

Always pathological

Question 351

Causes of jaundice in the first 24h

Answer 351

Rhesus disease ABO disease Hereditary spherocytosis G6PDD

Question 352

Jaundice 2-14d

Answer 352

Common and usually physiological, commonly seen in breast fed babies

Question 353

Causes of prolonged jaundice (\>14d)

Answer 353

Biliary atresia Hypothyroidism Galactosaemia HTI Breast milk jaundice Congenital infections e.g. CMV, toxoplasmosis

Question 354

Raised conjugated bilirubin in prolonged jaundice?

Answer 354

Could indicate biliary atresia which requires urgent surgical intervention

Question 355

Components of a prolonged jaundice screen

Answer 355

Conjugated and unconjugated bilirubin DAAT TFTs FBC and blood film urine for MC&S and reducing sugars U&Es LFTs

Question 356

Features of growing pains

Answer 356

A common presentation in General Practice is a child complaining of pain in the legs with no obvious cause. Such presentations, in the absence of any worrying features, are often attributed to 'growing pains'. This is a misnomer as the pains are often not related to growth - the current term used in rheumatology is 'benign idiopathic nocturnal limb pains of childhood' Growing pains are equally common in boys and girls and occur in the age range of 3-12 years. Features of growing pains never present at the start of the day after the child has woken no limp no limitation of physical activity systemically well normal physical examination motor milestones normal symptoms are often intermittent and worse after a day of vigorous activity

Question 357

Daniel is a newborn who is having his baby check done by nurse Karen, who notices that he has microcephaly with a prominent occiput, low set ears, micrognathia, palpebral fissures and wide spaced eyes. What genetic disorder are these features suggestive of? Edward's syndrome Down's syndrome Turner's syndrome Noonan syndrome Angelman syndrome

Answer 357

The correct answer for this question is Edward's syndrome. All of the aforementioned characteristics can be present in Edward's syndrome. Furthermore, individual's with Edward's syndrome can also have: Ptosis Rocker bottom feet Undescended testes

Question 358

Risk factors for SIDS

Answer 358

Most common at 3m of age Prematurity Parental smoking Hyperthermia Sleeping prone Male sex Multiple births Bottle feeding Social classes IV and V Maternal drug use Winter Following cot death, siblings should be screened for potential sepsis and inborn errors of metabolism

Question 359

Features of Achondroplasia

Answer 359

Achondroplasia is an autosomal dominant disorder associated with short stature. It is caused by a mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene. This results in abnormal cartilage giving rise to: short limbs (rhizomelia) with shortened fingers (brachydactyly) large head with frontal bossing midface hypoplasia with a flattened nasal bridge 'trident' hands lumbar lordosis

Question 360

Answer 360

Achondroplasia

Question 361

Features of kawasaki's disease

Answer 361

High-grade fever which lasts \>5d and is characteristically resistant to antipyretics Conjunctival injection Bright red, cracked lips Strawberry tongue Cervical lymphadenopathy Red plams of the hands and the soles of the feet which alter peel

Question 362

Answer 362

Kawasaki disease

Question 363

Mx of kawasaki

Answer 363

High dose aspirin IVIG Echocardiogram as screening test for coronary artery aneurysm

Question 364

Cx of kawasaki

Answer 364

Coronary artery aneurysm

Question 365

An 8-year-old boy presents with increasing jaundice over the past week. He was recently treated with nitrofurantoin for a simple urinary tract infection. On examination he is obviously jaundiced, and he is looking pale and breathless. Investigation results are as follows: Hb58 g/l Platelets250 \* 109/l WBC6.5 \* 109/l A blood films demonstrates red cell fragments and Heinz bodies. What is the most likely diagnosis? Pyruvate kinase deficiency Sickle cell disease Glucose-6-phosphate dehydrogenase deficiency Beta-thalassaemia Hereditary spherocytosis

Answer 365

Glucose-6-phosphate dehydrogenase deficiency is an X linked disorder affecting red cell enzymes. It results in a reduced ability of the red cells to respond to oxidative stress. Therefore, red cells have a shorter life span and are more susceptible to haemolysis, particularly in response to drugs (e.g. nitrofurantoin), infection, acidosis and certain dietary agents (e.g. fava beans). The red cell fragments, Heinz bodies and anaemia confirm a haemolytic anaemia.

Question 366

Features of G6PD

Answer 366

Features neonatal jaundice is often seen intravascular haemolysis gallstones are common splenomegaly may be present Heinz bodies on blood films

Question 367

Dx of G6PD

Answer 367

Enzyme assay

Question 368

Drugs thought to be safe in G6PD

Answer 368

Some drugs thought to be safe penicillins cephalosporins macrolides tetracyclines trimethoprim

Question 369

Inheritance of G6PD

Answer 369

X-linked recessive

Question 370

Inheritance of HS

Answer 370

Male+female AD

Question 371

Fraser guidelines

Answer 371

The following points should be fulfilled: the young person understands the professional's advice the young person cannot be persuaded to inform their parents or allow the professional to contact them on their behalf the young person is likely to begin, or continue having, sexual intercourse with or without contraceptive treatment unless the young person receives contraceptive treatment, their physical or mental health, or both, is likely to suffer the young person's best interests require them to receive contraceptive advice or treatment with or without parental consent

Question 372

A 6-year-old boy is reviewed in clinic due to nocturnal enuresis. His mother has tried using a star-chart but unfortunately this has not resulted in any significant improvement. Of the following options, what is the most appropriate initial management strategy? Enuresis alarm Trial of oral desmopressin Trial of imipramine Trial of intranasal desmopressin Restrict fluids in the afternoon and evening

Answer 372

Restricting fluids is not recommended advice - Clinical Knowledge Summaries suggest: 'Do not restrict fluids. The child should have about eight drinks a day, spaced out throughout the day, the last one about 1 hour before bed.' Enuresis alarm

Question 373

Def: enuresis

Answer 373

Involuntary discharge of urine by day or night or both in any child aged \>5 in the absence of congenital or acquired defects of the nervous system of the urinary tact Can be primary or secondary (dry for at least 5 months before)

Question 374

Mx of Nocturnal enuresis

Answer 374

Look for possible underlying causes/triggers: constipation, DM, UTI Advise on fluid intake Reward systems e.g. star charts Enuresis alarm for children \<7 Desmopressin may be used if \>7y particulalry if ST control is needed or an enuresis alarm has been ineffective

Question 375

Mx of meningitis

Answer 375

ABCD approach 1. Antibiotics 2. Steroids if \>1m and H influenzae, dexamehtasone 3. Fluids e.g. colloid 4. Cerebral monitoring 5. PH notification and antibiotic prophlyaxis of contacts- rifampicin

Question 376

Antibiotics in childhood meningitis

Answer 376

\<3m: IV amoxicillin and IV cefotaxime \>3m: IV cefotaxime

Question 377

Used for antibiotic prophylaxis of contacts to child with meningitis?

Answer 377

Rifampicin

Question 378

For patients with meningococcal septicaemia, LP?

Answer 378

Is contraindicated, blood cultures and PCR for meningococcus should be obtained

Question 379

Contraindications to LP

Answer 379

Signs of raised ICP Focal neurology Papilloedema Bulging of the fontanelle DIC Signs of cerebral herniation

Question 380

Henry is a 29 week premature baby who was born 2 weeks ago. Over the past week it has been noted that he has had bloody stool, abdominal distension and has not been feeding well. Physical examination reveals an increased abdominal girth with reduced bowel sounds. Abdominal X-ray shows dilated asymmetrical bowel loops and bowel wall oedema. What is the likely diagnosis? ## Footnote Intussusception Inflammatory bowel disease Pyloric stenosis Hirschsprung's disease Necrotising enterocolitis

Answer 380

The correct answer for this question is necrotising enterocolitis.

Question 381

AXR findings in NEC

Answer 381

dilated bowel loops (often asymmetrical in distribution) bowel wall oedema pneumatosis intestinalis (intramural gas) portal venous gas pneumoperitoneum resulting from perforation air both inside and outside of the bowel wall (Rigler sign) air outlining the falciform ligament (football sign)

Question 382

Features of NEC

Answer 382

Necrotising enterocolitis is one of the leading causes of death among premature infants. Initial symptoms can include feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration, perforation and peritonitis.

Question 383

You see a worried mum with her 6 month old baby boy. She is concerned that his skull shape is not normal. His development and birth have been normal and there are no conditions in the family. On examination his head circumference is at the 40th centile with his height and weight at the 30th centile. His occiput is flattened on the left, his left ear mildly protruding forward and his left forehead more prominent than the right. No other abnormality is detected. What is the most appropriate management? Urgent referral to neurosurgery Suggest buying an infant helmet Arrange an MRI scan Routine referral to community child health clinic Reassurance

Answer 383

Plagiocephaly is more common since there have been campaigns to encourage babies to sleep on their back to reduce the risk of sudden infant death syndrome (SIDS). Plagiocephaly is a skull deformity producing unilateral occipital flattening, which pushes the ipsilateral forehead ear forwards producing a 'parrallelogram' appearance. The vast majority improve by age 3-5 due to the adoption of a more upright posture. Helmets are not usually recommended as there was no significant difference between groups in a randomised controlled trial. Turning the cot around may help the child look the other way and take the pressure off the one side. Other simple methods include giving the baby time on their tummy during the day, supervised supported sitting during the day, and moving toys/ mobiles around in the cot to change the focus of attention. Ensure all advice is in line with prevention of SIDS.

Question 384

Plagiocephaly

Answer 384

parallelogram shaped head the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the 'Back to Sleep' campaign

Question 385

Craniosynostosis

Answer 385

premature fusion of skull bones

Question 386

A 4-year-old boy was discharged from the hospital six weeks ago after an episode of viral gastroenteritis. He now has 4-5 loose stools each day which has been present for the past four weeks. What is the most likely diagnosis? Coeliac disease Inflammatory bowel disease Secondary bacterial infection Lactose intolerance Clostridium difficile infection

Answer 386

Transient lactose intolerance is a common complication of viral gastroenteritis. Removal of lactose from the diet for a few months followed by a gradual reintroduction usually resolves the problem.

Question 387

Gastroenteritis

Answer 387

main risk is severe dehydration most common cause is rotavirus - typically accompanied by fever and vomiting for the first 2 days. The diarrhoea may last up to a week treatment is rehydration

Question 388

Causes of chronic diarrhoea in infants

Answer 388

most common cause in the developed world is cows' milk intolerance toddler diarrhoea: stools vary in consistency, often contain undigested food coeliac disease post-gastroenteritis lactose intolerance

Question 389

Which one of the following statements regarding cerebral palsy is incorrect? It is the most common cause of major motor impairment in children Less than 5% of children will have epilepsy It affects 2 in 1,000 live births 20% of children have hearing impairment Postnatal factors account for 10% of cases

Answer 389

Around 30% of children with cerebral palsy have epilepsy

Question 390

Def: Cerebal palsy

Answer 390

Cerebral palsy may be defined as a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain. It affects 2 in 1,000 live births and is the most common cause of major motor impairment

Question 391

Possible manifestations of cerebal palsy

Answer 391

Abnormal tone in early infancy Delayed motor milestones Abnormal gait Feeding difficulties

Question 392

Non-motor problems associated with cerebal palsy

Answer 392

learning difficulties (60%) epilepsy (30%) squints (30%) hearing impairment (20%)

Question 393

Causes of cerebal palsy

Answer 393

antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV) intrapartum (10%): birth asphyxia/trauma postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma

Question 394

Classification of cerebal palsy

Answer 394

Spastic (70%0: hemiplegia, diplegia or quadriplegia Dyskinetic Ataxic Miced

Question 395

Mx of cerebal palsy

Answer 395

MDT Spasticity: oral diazepam, oral and intrathecal baclofen, botulinum toxin type A. orthopaedic surgery Anticonvulsants, analgesia PRN

Question 396

What is the most common cause of nappy rash

Answer 396

Irritant dermatitis

Question 397

Irritant dermatitis

Answer 397

The most common cause, due to irritant effect of urinary ammonia and faeces Creases are characteristically spared

Question 398

Answer 398

Irritant dermatitis

Question 399

Candida dermatitis

Answer 399

Typically an erythematous rash which involve the flexures and has characteristic satellite lesions

Question 400

Answer 400

Typically an erythematous rash which involve the flexures and has characteristic satellite lesions

Question 401

Seborrhoeic dermatitis

Answer 401

Erythematous rash with flakes. May be coexistent scalp rash

Question 402

Mx Nappy rash

Answer 402

General management points ## Footnote disposable nappies are preferable to towel nappies expose napkin area to air when possible apply barrier cream (e.g. Zinc and castor oil) mild steroid cream (e.g. 1% hydrocortisone) in severe cases

Question 403

A 1-year-old girl is investigated for recurrent urinary tract infections. A micturating cystourethrogram is ordered: What does this image demonstrate? Vesicoureteric reflux Horseshoe kidney Paediatric urolithiasis Duplex collecting system Isolated right-sided hydronephrosis

Answer 403

This image demonstrates grade V vesicoureteric reflux - gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity. A DMSA scan is needed to identify renal scarring.

Question 404

Def: VUR

Answer 404

Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into the ureter and kidney. It is relatively common abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI

Question 405

Pathophysiology of VUR

Answer 405

Pathophysiology of VUR ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle therefore shortened intramural course of ureter vesicoureteric junction cannot therefore function adequately

Question 406

Grade I VUR

Answer 406

Into uretur only, no dilatation

Question 407

Grade II VUR

Answer 407

Into renal pelvis on micturition, no dilatation

Question 408

Grade III VUR

Answer 408

Mild/moderate dilatation of the uretur, renal pelvis and calyces

Question 409

Grade IV VUR

Answer 409

Dilatation of the renal pelvis and calyces with moderate ureteral tortuosity

Question 410

Grade V VUR

Answer 410

Gross dilatation of the uretur, pelvis and calyces with ureteral tortuosity

Question 411

Ix in VUR

Answer 411

Normally following a micturating cystourethrogram DMSA scan may be performed to look for renal scarring

Question 412

Features of bronchiolitis

Answer 412

Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases. SIGN released guidelines on bronchiolitis in 2006. Please see the link for more details. Epidemiology most common cause of a serious lower respiratory tract infection in \< 1yr olds (90% are 1-9 months, with a peak incidence of 3-6 months). Maternal IgG provides protection to newborns against RSV higher incidence in winter

Question 413

coryzal symptoms (including mild fever) precede: dry cough increasing breathlessness wheezing, fine inspiratory crackles (not always present) feeding difficulties associated with increasing dyspnoea are often the reason for hospital admission

Answer 413

Bronchiolitis

Question 414

Pathogens in bronchiolitis

Answer 414

respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases other causes: mycoplasma, adenoviruses may be secondary bacterial infection more serious if bronchopulmonary dysplasia (e.g. Premature), congenital heart disease or cystic fibrosis

Question 415

Def: Surfactant lung disease

Answer 415

Surfactant deficient lung disease (SDLD, also known as respiratory distress syndrome and previously as hyaline membrane disease) is a condition seen in premature infants. It is caused by insufficient surfactant production and structural immaturity of the lungs

Question 416

Risk of SDLD at 26-28w

Answer 416

50%

Question 417

Risk of SDLD at 30-31w

Answer 417

25%

Question 418

Other risk factors apart from gestation for SDLD

Answer 418

Male sex Diabetic mothers C sec Second born of premature twins

Question 419

Clinical features of SDLD

Answer 419

Tachypnoea Intercostal recession Expiratory grunting Cyanosis

Question 420

Mx of SDLD

Answer 420

Maternal corticosteroids O2 Assisted ventilation Exogenous surfactant given via endotracheal tube

Question 421

A 15-year-old boy presents to his GP complaining of knee pain for one week. He has no significant past medical history. Which of the following would make a diagnosis of Osgood-Schlatter disease more likely? Bilateral knee pain. Sudden onset of symptoms and acutely painful. Knee pain isolated to the posterior aspect of the knee joint. Pain relieved by rest and made worse by kneeling and activity, such as running or jumping. Locking of the knee on movement.

Answer 421

Osgood-Schlatter disease may be diagnosed on the basis of clinical features alone. This age group (adolescent) is the most likely age to suffer from this condition and is localized to the tibial tuberosity. Typically, pain is: Unilateral (but may be bilateral in up to 30% of people). Gradual in onset and initially mild and intermittent, but may progress to become severe and continuous. Relieved by rest and made worse by kneeling and activity, such as running or jumping.

Question 422

Chondromalacia patellae

Answer 422

Softening of the cartilage of the patella Common in teenage girls Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting Usually responds to physiotherapy

Question 423

Osgood-Schlatter disease | (tibial apophysitis)

Answer 423

Seen in sporty teenagers Pain, tenderness and swelling over the tibial tubercle

Question 424

Osteochondritis dissecans

Answer 424

Pain after exercise Intermittent swelling and locking

Question 425

Patellar subluxation

Answer 425

Medial knee pain due to lateral subluxation of the patella Knee may give way

Question 426

Patellar tendonitis

Answer 426

More common in athletic teenage boys Chronic anterior knee pain that worsens after running Tender below the patella on examination

Question 427

When is Guthrie test performed?

Answer 427

5-9d of life

Question 428

What conditions are included on Guthrie test?

Answer 428

Congenital hypothyroidism CF PKU SCD MCADD

Question 429

Features of strawberry naevi

Answer 429

Strawberry naevi (capillary haemangioma) are usually not present at birth but may develop rapidly in the first month of life. They appear as erythematous, raised and multilobed tumours. Typically they increase in size until around 6-9 months before regressing over the next few years (around 95% resolve before 10 years of age). Common sites include the face, scalp and back. Rarely they may be present in the upper respiratory tract leading to potential airway obstruction Capillary haemangiomas are present in around 10% of white infants. Female infants, premature infants and those of mothers who have undergone chorionic villous sampling are more likely to be affected

Question 430

Cx of strawberrys naevi

Answer 430

Mechanical: e.g. obstructing visual fields or airway Bleeding Ulceration Thrombocytopenia

Question 431

Mx of strawberry naevi

Answer 431

Most spontaneously resolve If treatment required can use propranolol

Question 432

What is a cavernous haemangioma?

Answer 432

Deep capilalry haemangioma

Question 433

Answer 433

Strwaberry naevus

Question 434

Cavernous haemangioma

Answer 434

Cavernous hemangioma, also calledcavernous angioma, or cerebral cavernoma (when referring to presence in the brain)[1] is a type of blood vessel malformation or hemangioma, where a collection of dilated blood vessels form abenign tumor. Because of this malformation, blood flow through the cavities, or caverns, is slow. Additionally, the cells that form the vessels do not form the necessary junctionswith surrounding cells. Also, the structural support from the smooth muscle is hindered, causing leakage into the surrounding tissue. It is the leakage of blood, known as a hemorrhage from these vessels that causes a variety of symptoms known to be associated with this disease.

Question 435

What are the innocent murmurs heard in children?

Answer 435

Ejection murmurs Venous hum Still's murmur

Question 436

Ejection murmur

Answer 436

Due to turbulent blood flow at the outflow tract of the heart

Question 437

Venous hum

Answer 437

Heard as a continuous blowing noise just below the clavicles Due to turbulent blood flow in the great veins

Question 438

Still's murmur

Answer 438

Low-pitched sound heard at the lower left sternal edge

Question 439

Characteristics of an innocent murmur

Answer 439

Soft blowing murmur in the pulmonary area or short buzzing murmur in the aortic area May vary with posture No radiation No diastolic component No thrill No added sounds e.g. clicks Asymptomatic child No other abnormality

Question 440

A 2-year-old boy is brought to the surgery by his mother with earache and pyrexia. On examination of the precordium a murmur is heard. Which one of the following characteristics is not consistent with an innocent murmur? Short buzzing murmur in the aortic area Soft-blowing murmur in the pulmonary area Varies with posture Diastolic murmur Continuous blowing noise heard just below the clavicles

Answer 440

Diastolic murmur

Question 441

Draw neonatal resscitation

Answer 441

Question 442

Draw basic PLS

Answer 442

Question 443

Ratio of chest compression in neonate?

Answer 443

3:1

Question 444

Ratio of chest compressions in paediatric

Answer 444

5 initial breaths then 15:1

Question 445

Def: Perthes disease

Answer 445

Perthes disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head Perthes disease is 5 times more common in boys. Around 10% of cases are bilateral

Question 446

Features of Perthes disease

Answer 446

Hip pain: develops progressively over a few weeks Limp Stiffness and reduced range of movement XR: early changes include joint space widening. Decreased femoral head size/flattening

Question 447

Cx of Perthes

Answer 447

Osteoarthritis Premature fusion of the growth plates

Question 448

Answer 448

Perthes disease Bilateral avascular necrosis of the femoral heads

Question 449

Draw the nephritic and the nephrotic syndromes

Answer 449

Question 450

Which one of the following types of glomerulonephritis is most characteristically associated with streptococcal infection in children? Focal segmental glomerulosclerosis Diffuse proliferative glomerulonephritis Membranous glomerulonephritis Mesangiocapillary glomerulonephritis Rapidly progressive glomerulonephritis

Answer 450

Diffuse proliferative glomerulonephritis

Question 451

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis

Answer 451

rapid onset, often presenting as acute kidney injury causes include Goodpasture's, ANCA positive vasculitis

Question 452

IgA nephropathy - aka Berger's disease, mesangioproliferative GN

Answer 452

typically young adult with haematuria following an URTI

Question 453

Diffuse proliferative glomerulonephritis

Answer 453

classical post-streptococcal glomerulonephritis in child presents as nephritic syndrome / acute kidney injury most common form of renal disease in SLE

Question 454

Membranoproliferative glomerulonephritis (mesangiocapillary)

Answer 454

type 1: cryoglobulinaemia, hepatitis C type 2: partial lipodystrophy

Question 455

Minimal change disease

Answer 455

typically a child with nephrotic syndrome (accounts for 80%) causes: Hodgkin's, NSAIDs good response to steroids

Question 456

Membranous glomerulonephritis

Answer 456

presentation: proteinuria / nephrotic syndrome / chronic kidney disease cause: infections, rheumatoid drugs, malignancy 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop chronic kidney disease

Question 457

Focal segmental glomerulosclerosis

Answer 457

may be idiopathic or secondary to HIV, heroin presentation: proteinuria / nephrotic syndrome / chronic kidney disease

Question 458

Features of absence seizures

Answer 458

Absence seizures (petit mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old and girls are affected twice as commonly as boys Features absences last a few seconds and are associated with a quick recovery seizures may be provoked by hyperventilation or stress the child is usually unaware of the seizure they may occur many times a day EEG: bilateral, symmetrical 3Hz spike and wave pattern

Question 459

Mx of absence seizures

Answer 459

sodium valproate and ethosuximide are first-line treatment good prognosis - 90-95% become seizure free in adolescence

Question 460

Live attenuated vaccines

Answer 460

BCG measles, mumps, rubella (MMR) influenza (intranasal) oral rotavirus oral polio yellow fever oral typhoid\*

Question 461

A mother brings her 3-year-old child in to receive the DTP booster. Which one of the following would make it inappropriate to give the vaccination today? Being below the 2nd centile for weight Family history of allergy to DTP Recent onset of a seizure disorder currently being investigated Planned general anaesthesia in 2 weeks time Being born at 29 weeks gestation

Answer 461

DTP: vaccination should be deferred in children with an evolving or unstable neurological condition

Question 462

General contraindications to immunisation

Answer 462

confirmed anaphylactic reaction to a previous dose of a vaccine containing the same antigens confirmed anaphylactic reaction to another component contained in the relevant vaccine (e.g. egg protein)

Question 463

Situations where vaccines should be delayed

Answer 463

febrile illness/intercurrent infection

Question 464

Contraindications to live vaccines

Answer 464

pregnancy immunosuppression

Question 465

What are the most common causes of hearing problems in children?

Answer 465

Conductive secretory otitis media Down's syndrome\* Sensorineural hereditary - Usher syndrome, Pendred syndrome, Jervell-Lange-Nielson syndrome, Wardenburg syndrome congenital infection e.g. rubella acquired - meningitis, head injury cerebral palsy perinatal insult

Question 466

16-year-old boy presents to the emergency room with a history of groin pain for the past three hours. He has associated nausea and has vomited three times. He reports that he recently had unprotected vaginal sex. On examination there is tenderness and swelling of the scrotum and left testicle, with absence of the cremaster reflex on the left side. Elevation of the affected testicle causes increased pain. What is the most likely diagnosis? Torsion of the hydatid of Morgagni Strangulated inguinal hernia Epididymitis Testicular torsion Hydrocoele

Answer 466

Testicular torsion occurs when the testis turns on the remnant of the processus vaginalis thereby restricting blood flow. It usually presents with acutely severe testicular pain often with associated nausea and vomiting. There may be swelling of the testis with overlying erythema. The cremaster reflex may also be absent on the affected side. Elevation of the testicle often results in worsening of the pain. Although this patient recently had unprotected sex, the history is less suggestive of epididymitis. With epididymitis we would expect urinary symptoms. In addition, elevation of the testes often relieves the pain (Prehn's sign positive).

Question 467

Ddx in acute scrotal disorder

Answer 467

Torsion Irreducible inguinal hernia Epididymitis

Question 468

What is a positive Prehn's sign

Answer 468

Elevation of the testes relieving pain. Seen in epididymitis

Question 469

Infants and nappies

Answer 469

Should have at least 6 heavy wet nappies in 24h

Question 470

Answer 470

Molluscum contagiosum

Question 471

Typical presentation of molluscum contagiosum

Answer 471

Typically, molluscum contagiosum presents with characteristic pinkish or pearly white papules with a central umbilication, which are up to 5 mm in diameter. Lesions appear in clusters in areas anywhere on the body (except the palms of the hands and the soles of the feet). In children, lesions are commonly seen on the trunk and in flexures, but anogenital lesions may also occur. In adults, sexual contact may lead to lesions developing on the genitalia, pubis, thighs, and lower abdomen. Rarely, lesions can occur on the oral mucosa and on the eyelids.

Question 472

At what age do the majority of children achieve day and night time urinary continence? 2-3 years old 3-4 years old 4-5 years old 5-6 years old 6-7 years old

Answer 472

The majority of children achieve day and night time continence by 3 or 4 years of age

Question 473

A jittery and hypotonic baby may suggest

Answer 473

neonatal hypoglycaemia.

Question 474

You are called to the post natal ward to review an 8 hour old baby born by elective caesarian section at 39 weeks gestation. After reading the case notes you discover the use of maternal labetalol for high blood pressure. On examination the baby appears jittery and hypotonic. What is the most appropriate next step? Record temperature and ensure adequately wrapped Perform full septic screen Measure blood glucose levels Start empirical antibiotics for early onset sepsis Re-examine after next feed

Answer 474

A jittery and hypotonic baby may suggest neonatal hypoglycaemia. The use of maternal labetalol is a risk factor and these babies must have their blood glucose measured. Neonatal abstinence syndrome may also present in this way and so the use of maternal opiates or illicit drug use in pregnancy should also be ascertained.

Question 475

Causes of neonatal hypoglycaemia

Answer 475

Maternal DM Prematurity IUGR Hypothermia Neonatal sepsis Inborn errors of metbolism Nesidioblastosis

Question 476

Nesidioblastosis

Answer 476

Nesidioblastosis is a controversial medical term for hyperinsulinemic hypoglycemiaattributed to excessive function ofpancreatic beta cells with an abnormalmicroscopic appearance. The term was coined in the first half of the 20th century. The abnormal histologic aspects of the tissue included the presence of islet cell enlargement, islet cell dysplasia, beta cells budding from ductal epithelium, and islets in apposition to ducts.

Question 477

Developmental milestones: speech and hearing 3 months

Answer 477

Quietens to parents voice Turns towards sound Squeals

Question 478

Developmental milestones: speech and hearing 6m

Answer 478

Double syllables: adah, erleh

Question 479

Developmental milestones: speech and hearing 9m

Answer 479

Mama, Dada understands no

Question 480

Developmental milestones: speech and hearing 12m

Answer 480

Responds to own name

Question 481

Developmental milestones: speech and hearing 12-15m

Answer 481

Knows about 2-6 words Refer at 18m Understands simple commands

Question 482

Developmental milestones: speech and hearing 2y

Answer 482

Combine 2 words Points to parts of the body

Question 483

Developmental milestones: speech and hearing 2.5t

Answer 483

Vocabulary of 200w

Question 484

Developmental milestones: speech and hearing 3y

Answer 484

Talks in short sentences (3-5w) Asks what and who questions Identifies colours Counts to 10

Question 485

Developmental milestones: speech and hearing 4y

Answer 485

Asks why, when and how questions

Question 486

What are the criteria for sending a child with an acute limp for urgen assesment?

Answer 486

\<3 \>8 with painful or restricted hip movements (in particular internal rotation) to exclude SUFE Is unable to weight bear Has fever and or red flags suggesting serious pathology: pain waking them at night, fatigue, anorexia, weight loss, night sweats In severe pain, agitated or has reduced peripheral pulses or muscle weakness that may indicate neurovascular compromise or impending compartment syndrome ?Maltreatment

Question 487

Transient synovitis

Answer 487

Acute onset Usually accompanies viral infections, but the child is well or has a mild fever More common in boys, aged 2-12 years

Question 488

Septic arthritis/osteomyelitis

Answer 488

Unwell child, high fever

Question 489

Juvenile idiopathic arthritis

Answer 489

Limp may be painless

Question 490

Slipped upper femoral epiphysis

Answer 490

10-15 years - Displacement of the femoral head epiphysis postero-inferiorly

Question 491

On routine antenatal swabs, a mother is found to be colonised with Group B Streptococcus. However, she did not receive adequate intrapartum antibiotic prophylaxis and she delivers a healthy baby girl by vaginal delivery. Her baby does not require any resuscitation and remains well in the post natal ward. The mother is eager for discharge home. What is the most appropriate course of action with regards to her child? ## Footnote Intravenous antibiotics for 24 hours Check C-Reactive protein levels and take blood cultures Discharge if no suspicion of infection Perform routine 6 hour post natal check and discharge with community midwife follow up. Regular observations for 24 hours

Answer 491

Maternal colonisation with group B streptococcus is a minor risk factor for early onset sepsis in the newborn. Newborns with only one minor risk factor for early onset sepsis should remain in hospital for at least 24 hours with regular observations. Two or more minor risk factor or one red flag warrant empirical antibiotic therapy with Benzylpenicillin and Gentamicin and a full septic screen.

Question 492

Red flags for GBS infection

Answer 492

Suspected or confirmed infection in another baby in the case of a multiple pregnancy Parenteral antibiotic treatment given to the woman for confirmed or suspected invasive bacterial infection (such as septicaemia) at any time during labour, or in the 24-hour periods before and after the birth [This does not refer to intrapartum antibiotic prophylaxis] Respiratory distress starting more than 4 hours after birth Seizures Need for mechanical ventilation in a term baby Signs of shock

Question 493

A 9-year-old girl is brought to surgery as her mother is concerned that she is too fat. This has now been a problem for over two years and mum feels this is holding her back at school. What is the most appropriate method to ascertain how obese she is? Body mass index Body mass index percentile adjusted to age and gender Weight plotted on percentile chart Mother's perception Waist circumference

Answer 493

Defining obesity is more difficult in children than adults as body mass index (BMI) varies with age. BMI percentile charts are therefore needed to make an accurate assessment. Recent NICE guidelines suggest to use 'UK 1990 BMI charts to give age- and gender-specific information'

Question 494

Assessment of obese child

Answer 494

NICE recommend consider tailored clinical intervention if BMI at 91st centile or above. consider assessing for comorbidities if BMI at 98th centile or above

Question 495

Causes of obesity in children

Answer 495

Lifestyle growth hormone deficiency hypothyroidism Down's syndrome Cushing's syndrome Prader-Willi syndrome

Question 496

Consequences of obesity in children

Answer 496

Consequences of obesity in children orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in bowing of the legs), musculoskeletal pains psychological consequences: poor self-esteem, bullying sleep apnoea benign intracranial hypertension long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease

Question 497

Features of Acne vulgaris

Answer 497

Acne vulgaris is a common skin disorder which usually occurs in adolescence. It typically affects the face, neck and upper trunk and is characterised by the obstruction of the pilosebaceous follicle with keratin plugs which results in comedones, inflammation and pustules. Epidemiology affects around 80-90% of teenagers, 60% of whom seek medical advice acne may also persist beyond adolescence, with 10-15% of females and 5% of males over 25 years old being affected

Question 498

Pathophysiology of acne vulgaris

Answer 498

follicular epidermal hyperproliferation resulting in the formation of a keratin plug. This in turn causes obstruction of the pilosebaceous follicle. Activity of sebaceous glands may be controlled by androgen, although levels are often normal in patients with acne colonisation by the anaerobic bacterium Propionibacterium acnes inflammation

Question 499

What are the referral points in developmental problems

Answer 499

Referral points doesn't smile at 10 weeks cannot sit unsupported at 12 months cannot walk at 18 months

Question 500

Hand preference before 12m?

Answer 500

Is abnormal and may indicate cerebal palsy

Question 501

Gross motor problems most common causes?

Answer 501

Variant of normal CP Neuromuscular disorders

Question 502

Speech and language problems, causes

Answer 502

Check hearing Environmental deprivation General developmental delay

Question 503

Def: febrile convulsions

Answer 503

Febrile convulsions are seizures provoked by fever in otherwise normal children. They typically occur between the ages of 6 months and 5 years and are seen in 3% of children

Question 504

Clinical features of febrile convulsions

Answer 504

usually occur early in a viral infection as the temperature rises rapidly seizures are usually brief, lasting less than 5 minutes may be generalised tonic or tonic-clonic

Question 505

Px following febrile convulsion

Answer 505

risk of further febrile convulsion = 1/3 (higher if family history) if recurrences, try teaching mother how to use rectal diazepam if no focal signs + lasts less than 30 minutes\* + single seizure then 1% risk of developing epilepsy in the \<1% who have all these features, risk of developing epilepsy is much higher (e.g. 50%)

Question 506

A newborn male baby is found to have an undescended right testicle during the routine 6-8 week examination. It is neither palpable in the scrotum or inguinal canal. What is the most appropriate management? Outpatient referral to urology to be seen within 4 weeks Review at 3 months Immediate referral to urology Arrange ultrasound abdomen and scrotum Review at 12 months

Answer 506

Undescended testicle - wait 6 months prior to referral If the testicle has not descended by around 3 months then referral should be considered for orchidopexy.

Question 507

Features of undescended testis

Answer 507

Undescended testis occurs in around 2-4% of term male infants., but is much more common if the baby is preterm. Around 25% of cases are bilateral Complications of undescended testis infertility torsion testicular cancer psychological Management orchidopexy: NICE CKS now recommend referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age. Surgical practices vary although the majority of procedures are performed at around 1 year of age

Question 508

What are the organisms which may colonise a patient with CF?

Answer 508

Staphylococcus aureus Pseudomonas aeruginosa Burkholderia cepacia\* Aspergillus

Question 509

Which one of the following best describes the emergency treatment of a child with severe croup? Oxygen + nebulised saline Oxygen + nebulised adrenaline Oxygen + nebulised salbutamol Oxygen + IM benzylpenicillin Oxygen + nebulised steroids

Answer 509

Oxygen + nebulised adrenaline Oral dexamethasone should also be given if the child is able to take it.

Question 510

A 6-week-old term infant has difficulty feeding due to increased breathlessness. As the on-call doctor you are called to review this baby. You witness the baby feeding and note she is pink and well perfused but sweating profusely with and increased respiratory rate. On examination you hear a soft pan-systolic murmur at the lower left sternal border. What is the most likely underlying pathology? Transient tachypnoea of the newborn Acute respiratory distress syndrome Heart failure Eisenmenger syndrome Infantile pneumonia

Answer 510

Heart failure typically presents in infants with symptoms of breathlessness worse on exertion (e.g. feeding), sweating, poor feeding and recurrent chest infections. On examination you should: examine the growth charts (?failure to thrive), examine for tachycardia, tachypnoea, murmurs and pre and post-ductal saturations. Heart failure may be due to duct dependant systemic circulations (\<2 weeks old) e.g. coarctation of the aorta or left-to-right shunts (\>2 weeks old) e.g. VSD as the pulmonary vasculature resistance begins to fall. In this case the baby has a large VSD causing decompensated heart failure. Cardiac lesions can be missed during the foetal anomaly scan and this baby would need a detailed foetal echocardiogram and discussion with the cardiac team on management strategies.

Question 511

What are the acyanotic congenital heart defects?

Answer 511

Acyanotic - most common causes ventricular septal defects (VSD) - most common, accounts for 30% atrial septal defect (ASD) patent ductus arteriosus (PDA) coarctation of the aorta aortic valve stenosis VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents later

Question 512

What are the most common causes of cyanotic congenital heart disease?

Answer 512

Cyanotic - most common causes tetralogy of Fallot transposition of the great arteries (TGA) tricuspid atresia pulmonary valve stenosis Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at around 1-2 months

Question 513

Factors which point towards child abuse include:

Answer 513

story inconsistent with injuries repeated attendances at A&E departments late presentation child with a frightened, withdrawn appearance - 'frozen watchfulness'

Question 514

Possible physical presentations of child abuse include:

Answer 514

bruising fractures: particularly metaphyseal, posterior rib fractures or multiple fractures at different stages of healing torn frenulum: e.g. from forcing a bottle into a child's mouth burns or scalds failure to thrive sexually transmitted infections e.g. Chlamydia, Gonorrhoea, Trichomonas

Question 515

Answer 515

Henoch Schonlein Purpura

Question 516

Features of HSP

Answer 516

Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Berger's disease). HSP is usually seen in children following an infection. Features palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs abdominal pain polyarthritis features of IgA nephropathy may occur e.g. haematuria, renal failure

Question 517

Mx of HSP

Answer 517

Analgesia for arthralgia Supportive treatment for nephropathy

Question 518

Px of HSP

Answer 518

Self-limiting condition, especially in children without renal involvement 1/3rd have a relapse

Question 519

An 18 month old child attends the paediatric assessment unit with his mother. He has been brought in as he has had a fever, barking cough and difficulty breathing at night. He has been diagnosed with croup and you have been asked to see him to review. After history and assessment you are confident there is no stridor or respiratory distress. What would your next step in management be? Give antibiotics Give oxygen Full ENT exam Give nebulised adrenaline Give oral dexamethasone

Answer 519

This child has mild croup, the severity of croup is based upon; respiratory rate, respiratory distress, heart rate, O2 saturations and exhaustion. Treatment of mild croup is oral dexamethasone 0.15mg/kg single dose and review. Systemic dexamethasone and nebulised adrenaline 5ml of 1:1000 are used in severe croup, alongside oxygen administration. Antibiotics should not be given unless an underlying bacterial infection is suspected. You should not perform an ENT exam due to the possibility of an epiglottis diagnosis.

Question 520

Features of foetal alcohol syndrome

Answer 520

Features short ­palpebral fissure thin vermillion border/hypoplastic upper lip smooth/absent filtrum learning difficulties microcephaly growth retardation epicanthic folds Baby may show symptoms of alcohol withdrawal at birth e.g. irritable, hypotonic, tremors

Question 521

A mother notices that her newborn boy has small eye openings, a small body and low-set ears. On examination the paediatrician also notes a flat philtre, a sunken nasal bridge, short palpebral fissures and a thin upper lip. What is the most likely cause? Diabetes Maternal alcohol abuse Group B Streptococcal infection Maternal Listeria Maternal opioid abuse

Answer 521

Fetal alcohol syndrome Maternal alcohol abuse during pregnancy. Presentation: IUGR, microcephaly, midfacial hypoplasia, micrognathia, smooth philtrum, microphthalmia, short palpebral fissures, thin upper lip, irritability, ADHD.

Question 522

What is the most common cause of DS and risk of recurrence?

Answer 522

94% non-disjunction

Question 523

What proportion of DS caused by Robertsonian translocation? Risk of recuccrence

Answer 523

5% (usually onto chromosome 14) 10-15% if mother is translocation carrier 2.5% if father is translocation carrier

Question 524

What are the chromosomal causes of DS

Answer 524

Non disjunction Translocation Mosaicism

Question 525

A mother asks for information following a recent admission of her 2-year-old son with a febrile convulsion. What is the chance of her son having a further febrile convulsion?

Answer 525

Febrile convulsions - risk of further convulsions = 30%

Question 526

An 11-year-old girl presents with a productive cough and fever. A chest x-ray is taken: Bilateral pneumothoraces Left lingula consolidation Dilated cardiomyopathy with pulmonary oedema Left humeral head fracture Left middle lobe consolidation

Answer 526

The loss of the left heart border is a classic sign of left lingula consolidation. There is no left middle lobe!

Question 527

A 10-year-old girl is admitted with shortness-of-breath and fatigue. A chest x-ray is performed on admission: Based on the x-ray findings, what is the most likely diagnosis? Inhaled foreign object Heart failure Asthma Cystic fibrosis Pneumonia

Answer 527

The x-ray shows dilated cardiomyopathy and features of pulmonary oedema including fluid in the horizontal fissure.

Question 528

Causes of snoring in children

Answer 528

Obesity Nasal problems: polyps, deviated septum, hypertrophic nasal turbinates Recurrent tonsilitis DS Hypothyroidism

Question 529

Disease not excluded from school

Answer 529

Conjunctivitis Fifth disease Roseola Infectious mononucleosis Head lice Threadwormsq

Question 530

Fifth disease

Answer 530

Erythema infectiosum or fifth disease is one of several possible manifestations of infection by parvovirus B19.[1] The disease is also referred to as slapped cheek syndrome, slapcheek, slap face orslapped face.[2][3] The name "fifth disease" comes from its place on the standard list ofrash-causing childhood diseases, which also includes measles (1st),scarlet fever (2nd), rubella (3rd), and Dukes' disease (4th), though the latter is no longer widely accepted as distinct.

Question 531

Roseola infantum

Answer 531

Roseola is a disease of children, generally under two years old.[1]Although it has been known to occur in eighteen-year-olds, whose manifestations are usually limited to a transient rash ("exanthem") that occurs following a fever of about three days' duration. It is caused by two human herpesviruses, human herpesvirus 6 (HHV-6) and human herpesvirus 7 (HHV-7), which are sometimes referred to collectively as Roseolovirus. There are two variants of HHV-6 (HHV-6a and HHV-6b) and studies in the US, Europe, Dubai and Japan have shown that exanthema subitum is caused by HHV-6b. This form of HHV-6 infects over 90% of infants by age 2. Research has shown that babies can be congenitally infected with HHV-6 via vertical transmission.[2] This has been shown to occur in 1% of children in the United States.[3][4]

Question 532

Excluded from school for 24h after commencing antibiotics

Answer 532

Scarlet fever

Question 533

School exclusion 4 d from onset of rash

Answer 533

Measles

Question 534

School exclusion 5d from onset of rash

Answer 534

Chickenpox

Question 535

School exclusion 5d from onset of swollen glands

Answer 535

Mumps

Question 536

School exclusion until 5d after commencing antibiotics

Answer 536

Whooping cough

Question 537

School exclusion 6d from onset of rash

Answer 537

Rubella

Question 538

School exclusion until symptoms have settled over 48h

Answer 538

D+V

Question 539

School exclusion until lesions have crusted over

Answer 539

Impetigo

Question 540

School exclusion until treated

Answer 540

Scabies

Question 541

School exclusion until recovered

Answer 541

IFV

Question 542

USS screening for DDH

Answer 542

Breech presentation is a risk factor for developmental dysplasia of the hip (DDH), so you should check that the baby has been referred for screening for this condition. The Department of Health advises that all babies that were breech at any point from 36 weeks (even if not breech by time of delivery), babies born before 36 weeks who had breech presentation, and all babies with a first degree relative with a hip problem in early life, should be referred for ultrasound of the hips. If one of a pair of twins is breech, both should be screened. Some Trusts also refer babies with other conditions including oligohydramnios, high birthweight, torticollis, congenital talipes calcaneovalgus and metatarsus adductus. Further details on screening for DDH can be found at the link below.

Question 543

For which one of the following indications is carbamazepine least likely to be a useful management option? Trigeminal neuralgia Absence seizures Bipolar disorder Temporal lobe epilepsy Complex partial seizures

Answer 543

Carbamazepine is generally ineffective in absence seizures

Question 544

Features of carbamazepine

Answer 544

Carbamazepine is chemically similar to the tricyclic antidepressant drugs. It is most commonly used in the treatment of epilepsy, particularly partial seizures, where carbamazepine remains a first-line medication. Other uses include neuropathic pain (e.g. trigeminal neuralgia, diabetic neuropathy) bipolar disorder Mechanism of action binds to sodium channels increases their refractory period

Question 545

Adverse effects of carbamazepine

Answer 545

Adverse effects P450 enzyme inducer dizziness and ataxia drowsiness headache visual disturbances (especially diplopia) Steven-Johnson syndrome leucopenia and agranulocytosis syndrome of inappropriate ADH secretion

Question 546

First sign of male puberty

Answer 546

Testicular growth at 10-15y/o Testicular volume \>4ml= onset of puberty Maximum height spurt at 14

Question 547

Normal changes during puberty

Answer 547

Gynaecomastia in boys Asymmetrical breast growth in girls Diffuse enlargement of the thyroid gland may be seen

Question 548

A 2 day old baby who was born by a ventouse delivery is noted to have a swelling on the left side of his head in the parietal region. His head appeared normal immediately after delivery. On examination, the baby is well and the swelling does not cross suture lines. The fontanelles and sutures appear normal. What is the most likely diagnosis? Subaponeurotic haematoma Caput succedeneum Craniosynostosis Skull fracture Cephalohaematoma

Answer 548

A cephalohaematoma appears as a swelling due to bleeding between the periosteum and the skull. It is most commonly noted in the parietal region and is associated with instrumental deliveries. The swelling usually appears 2-3 days following delivery and does not cross suture lines. It gradually resolves over a number of weeks.

Question 549

Draw difference between caput succedaneum and cepahlohaematoma

Answer 549

Question 550

Answer 550

Caput succedaneum

Question 551

Cephalohaematoma

Answer 551

Question 552

Answer 552

Question 553

A 9-year-old boy who has recently arrived from India presents with fever. On examination a grey coating is seen surrounding the tonsils and there is extensive cervical lymphadenopathy. What is the most likely diagnosis? Dengue fever Typhoid Paratyphoid Actinomycosis Diphtheria

Answer 553

Diphtheria

Question 554

Features of diptheria

Answer 554

Diphtheria is caused by the Gram positive bacterium Corynebacterium diphtheriae Pathophysiology releases an exotoxin encoded by a β-prophage exotoxin inhibits protein synthesis by catalyzing ADP-ribosylation of elongation factor EF-2 Diphtheria toxin commonly causes a 'diphtheric membrane' on tonsils caused by necrotic mucosal cells. Systemic distribution may produce necrosis of myocardial, neural and renal tissue

Question 555

Presentation of diptheria

Answer 555

Recent visitor to Eastern Europe/Russia/Asia Sore throat with a diptheric membrane Bulky cervical lymphadenoapthy Neuritis e.g. cranial nerves Heart block

Question 556

Answer 556

Dipthertitic membrane

Question 557

A baby born at 35 weeks gestations via normal vaginal delivery is found to be irritable 48 hours after birth and suffers a convulsion. There is no obvious head trauma or swellings. Which one of the following cranial injuries is most likely to have occurred? Caput succedaneum Cephalohaematoma Subaponeurotic haemorrhage Intraventricular haemorrhage Extradural haemorrhage

Answer 557

Caput succedaneum is caused by pressure on the fetal scalp during the birthing process. It results in a large oedematous swelling and bruising over the scalp. Treatment is not required as the swelling reduces over a few days. A cephalohaematoma may occur after a spontaneous vaginal delivery or following a trauma from the obstetric forceps or the ventouse. A haemorrhage results after the presidium is sheared from the parietal bone. The tense swelling is limited to the outline of the bone. It reduces over a few weeks - months. A Subaponeurotic haemorrhage, also known as a subgaleal haemorrhage is rare and is due to a traumatic birth. It may result in the infant losing large amounts of blood. An intracranial haemorrhage refers to subarachnoid, subdural or intraventricular haemorrhages. Subarachnoid haemorrhages are common and may cause irritability and even convulsions over the first 2 days of life. Subdural can following the use of forceps. Intraventricular haemorrhage mostly affects pre-term infants and can be diagnosed by ultrasound examinations. Extradural haemorrhage is unlikely to occur during the birthing process.

Question 558

Features of intraventricular haemorrhage

Answer 558

Haemorrhage into the ventricles Occurs in premature neonates, may occur spontaneously Blood may clot and occlue CSF flow, hydrocephalus may result. Vast majority occurs in frist 72h after birth

Question 559

Treatment of intraventricular haemorrhage

Answer 559

Supportive Most treatments have been trialled and shown not to be of benefit Hydrocephalus and rising ICP is an indication for shunting

Question 560

You are an FY1 on the paediatric ward round with your consultant. Whilst seeing a child that has been admitted with croup, the consultant you're with decides to quiz you on the pathophysiology. 'What is the most likely organism to cause croup?' Respiratory syncytial virus (RSV) Parainfluenza virus Pseudomonas aeruginosa Streptococcus pneumoniae Bordetella pertussis

Answer 560

Parainfluenza

Question 561

A 2-year-old boy presents with a harsh cough and pyrexia. His symptoms worsened overnight and on examination stridor is noted. Which one of the following interventions may improve his symptoms? Codeine linctus Humidified oxygen Nebulised salbutamol Oral erythromycin Oral dexamethasone

Answer 561

Oral dexamethasone

Question 562

A 5-year-old girl attends your GP surgery with her mother. She reports a five day history of a sore throat and fever. On examination you note a bright red tongue, flushed face and a rough dry erythematous rash on her neck. What is the most likely diagnosis? Measles Rubella Bordetella pertussis Kawasaki disease Scarlet fever

Answer 562

A strawberry tongue can be seen in both scarlet fever and Kawasaki disease. However given the history a diagnosis of scarlet fever is more likely.

Question 563

Lay rescuers vs two or more trained rescuers in PLS

Answer 563

30:2 vs 15:2 chest compressions to breaths

Question 564

Presenting features of CF

Answer 564

Neonatal period (20%): meconium ileus, prolonged jaundice Recurrent chest infections (40%) Malabsroption (30%): steatorrhoea, FTT Other featuers: liver disease Short stature DM Delayed puberty Rectal prolapse (due to bulky stools) Nasal polyps Male infertility, female subfertility

Question 565

Developmental milestones: fine motor and vision 3m

Answer 565

Reaches for object Holds rattle briefly if given to hand Visually alert, particularly to human faces Fixes and follows to 180 deg

Question 566

Developmental milestones: fine motor and vision 6m

Answer 566

Holds in palmar grasps Passes objects between hands Visually insatiable: looking around in every direction

Question 567

Developmental milestones: fine motor and vision 9m

Answer 567

Points with finger Early pincer

Question 568

Developmental milestones: fine motor and vision 12m

Answer 568

Good pincer grip Bangs toys together

Question 569

Developmental milestones: fine motor and vision Bricks 15m

Answer 569

Tower of 2

Question 570

Developmental milestones: fine motor and vision Bricks 18m

Answer 570

Tower of 3

Question 571

Developmental milestones: fine motor and vision Bricks 2y

Answer 571

Tower of 6

Question 572

Developmental milestones: fine motor and vision Bricks 3y

Answer 572

Tower of 9

Question 573

Developmental milestones: fine motor and vision drawing 18m

Answer 573

Circular scribble

Question 574

Developmental milestones: fine motor and vision drawing 2y

Answer 574

Copies vertical line

Question 575

Developmental milestones: fine motor and vision drawing 3y

Answer 575

Copies circle

Question 576

Developmental milestones: fine motor and vision drawing 4y

Answer 576

Copies cross

Question 577

Developmental milestones: fine motor and vision drawing 5y

Answer 577

Copies square and triangle

Question 578

Developmental milestones: fine motor and vision book 15m

Answer 578

Looks at book, pats page

Question 579

Developmental milestones: fine motor and vision book 18m

Answer 579

Turns pages several at a time

Question 580

Developmental milestones: fine motor and vision book 2y

Answer 580

Turns pages, one at a time

Question 581

Mx of chickenpox

Answer 581

Keep cool, trim nails Calamine lotion School exclusion Immunocompromised patients and newborns with peripartum exposure should receive VZIG. If chickenpox develops, IV aciclovir should be considered

Question 582

Cxs of chickenpox

Answer 582

Commonly: secondary bacterial infection of the lesioins Rarer: pneumonia encephalitis (cerebellar involvement) disseminated haemorrhagic chcikenpox arthritis, nephritis, pancreatitis

Question 583

Answer 583

Chest x-ray showing miliary opacities secondary to healed varicella pneumonia. Multiple tiny calcific miliary opacities noted throughout both lungs. These are of uniform size and dense suggesting calcification. There is no focal lung parenchymal mass or cavitating lesion seen.The appearances are characteristic for healed varicella pneumonia.

Question 584

Features of VZV

Answer 584

Chickenpox is caused by primary infection with varicella zoster virus. Shingles is reactivation of dormant virus in dorsal root ganglion Chickenpox is highly infectious spread via the respiratory route can be caught from someone with shingles infectivity = 4 days before rash, until 5 days after the rash first appeared\* incubation period = 10-21 days Clinical features (tend to be more severe in older children/adults) fever initially itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular systemic upset is usually mild

Question 585

Contraindications to MMR

Answer 585

severe immunosuppression allergy to neomycin children who have received another live vaccine by injection within 4 weeks pregnancy should be avoided for at least 1 month following vaccination immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are present)

Question 586

Adverse effects of MMR

Answer 586

malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days

Question 587

An 8-year-old boy presents with weakness and purple striae on his abdomen. On examination he is obese with a central fat distribution and is found to have facial plethora. He is also found to have a blood pressure of 130/85 mmHg and facial plethora. What is the most likely underlying cause? ACTH-secreting pituitary tumour Adrenal carcinoma Craniopharyngioma Congenital adrenal hyperplasia Ectopic adrenocorticotropin-producing tumour

Answer 587

The history is suggestive of Cushing's syndrome. In an 8-year old boy the commonest cause of Cushing's would be iatrogenic use of glucocorticoids. Out of the options above the most likely answer is an ACTH-secreting pituitary tumour.

Question 588

Eczema herpeticum

Answer 588

Eczema herpeticum describes a severe primary infection of the skin by herpes simplex virus 1 or 2. It is more commonly seen in children with atopic eczema. As it is potentially life threatening children should be admitted for IV aciclovir

Question 589

A 2-year-old child with a history of atopic eczema is brought to the local GP surgery. Her eczema is usually well controlled with emollients but her parents are concerned as the facial eczema has got significantly worse overnight. She now has painful clustered blisters on both cheeks, around her mouth on her neck. Her temperature is 37.9ºC. What is the most appropriate management? Advise paracetamol + emollients and reassure Admit to hospital Add hydrocortisone 1% Oral flucloxacillin Topical fusidic acid

Answer 589

Eczema herpeticum is a serious condition that requires IV antivirals

Question 590

IM benzylpenicillin for suspected meningococcal septicaemia in the community dose \<1y

Answer 590

300mg

Question 591

IM benzylpenicillin for suspected meningococcal septicaemia in the community dose 1-10y

Answer 591

600

Question 592

IM benzylpenicillin for suspected meningococcal septicaemia in the community dose \>10y

Answer 592

1200mg

Question 593

Def: precocious puberty

Answer 593

'development of secondary sexual characteristics before 8 years in females and 9 years in males' more common in females

Question 594

How can precocious puberty be classified?

Answer 594

Gonadotrophin dependant (central/true) Gonadotrophin independant

Question 595

Central precocious puberty

Answer 595

Due to premature access of the HPgonadal axis FSH and LH raised

Question 596

Pseudo precocious puberty

Answer 596

Due to excess sex hormones FSH and LH low

Question 597

Precocious puberty in males?

Answer 597

Uncommon, usually has an organic cause

Question 598

Bilateral testes enlargement in male with precocious puberty

Answer 598

Gonadotrophin release from intracranial lesion

Question 599

Unilateral enlargement of testes in precocious puberty

Answer 599

Gonadal tumour

Question 600

Small testes in precocious puberty

Answer 600

Adrenal cause (tumour or adrenal hyperplasia)

Question 601

Precocious puberty in females

Answer 601

Usually idiopathic or familial and follows normal sequence of puberty Organic causes are rare associated with rapid onset, neurological symptoms and signs and dissonance e.g. McCune Albright syndrome

Question 602

A mother brings her 9-year-old daughter into surgery. She has been having recurrent headaches. Which one of the following features of migraine is more common in children? Prolonged migraines (e.g. 24-48 hours) Strictly unilateral symptoms Hemiplegia Good response to metoclopramide Gastrointestinal disturbance

Answer 602

Nausea, vomiting and abdominal pain are common in children with migraine. Please rate this question:

Question 603

Migraines in children

Answer 603

Tend to be shorter-lasting Headache commonly bilateral GI disturbance more prominent

Question 604

Aura symptoms

Answer 604

motor weakness double vision visual symptoms affecting only one eye poor balance decreased level of consciousness.

Question 605

Minimal change disease

Answer 605

75% of cases of nephrotic syndrome in children

Question 606

Causes of nephrotic syndrome

Answer 606

Majority are idiopathic Cause found in 10-20% Drugs: NSAIDS, rifampicin HL, thymoma Infectious mononucleosis

Question 607

Pathophysiology of minimal change disease

Answer 607

T cell and cytokine mediated damage to the GBM-\> polyanion loss. Resultant reduciton of electrostatic charge \_\> increased glomerular permeability to serum albumin

Question 608

Featrues of minimal change disease

Answer 608

Nephrotic syndrome Normotension (HTN rare) Highly selective proteinuria: only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus Renal biopsy: electron micrsoscpy shows podocyte fusion

Question 609

Mx of minimal change disease

Answer 609

80% are steroids responsive Cyclophosphamide is the next step

Question 610

Px of minimal change disease

Answer 610

1/3rd have one episode 1/3rd have infrequent relapses 1/3rd have frequent relapses

Question 611

Prader Willi syndrome pathophysiology

Answer 611

Prader-Willi syndrome is an example of genetic imprinting where the phenotype depends on whether the deletion occurs on a gene inherited from the mother or father: Prader-Willi syndrome if gene deleted from father Angelman syndrome if gene deleted from mother Prader-Willi syndrome is associated with the absence of the active Prader-Willi gene on the long arm of chromosome 15. This may be due to: microdeletion of paternal 15q11-13 (70% of cases) maternal uniparental disomy of chromosome 15

Question 612

Features of PWS

Answer 612

Hypotonia during infancy Dysmorphic features Short features Hypogonadism and infertility LD Childhood obesity Behavioural problems in adolescence

Question 613

You are in a genetics clinic and explaining to a mother and father the reasoning why their son has Prader-Willi syndrome. What is the term we use to describe the mode of inheritance for Prader-Willi syndrome? Autosomal recessive Autosomal dominant Imprinting Pleiotropy Variable expressivity

Answer 613

Prader-Willi is an example of imprinting. For this disease to occur, the patient does not receive the gene from their father. The mother's gene may be normal, but that does not prevent the phenotype occurring. The phenotype consists of learning difficulties, hypotonia, obesity and the urge to eat. Autosomal recessive is when a person receive a defect gene from the mother and a defective gene from the father causing them to have the particular condition. An example of this is cystic fibrosis. Autosomal dominant refers to when a person only need to receive one defective gene to inherit a condition, this can be from the mother or father. An example of this would Huntington's disease. Pleiotropy refers to when one gene, when defective, causing two or more clinical effects that appear unrelated. Variable expressibility refers to when an inherited genetic defect causes different levels of clinical effect.

Question 614

A baby is born at term via vaginal delivery with no complications, however he is still not showing signs of breathing at one minute. Heart rate is \>100bpm, but he is floppy and cyanosed. What is the most appropriate next step in management? Call for anaesthetist to intubate the baby 5 mouth-to-mouth rescue breaths 5 breaths of oxygen via face mask Start chest compressions Suction airways

Answer 614

Airway suction should not be performed unless there is obviously thick meconium causing obstruction, as it can cause reflex bradycardia in babies. Chest compressions are not indicated, as the HR in this case is \>100bpm. CPR should only be commenced at a HR \< 60bpm. In cases where there are no signs of breathing and this is thought to be due to fluid in the lungs, five breaths should be given via a 250ml bag via face mask. This is a more effective and more hygienic method than using mouth-to-mouth in a hospital setting.

Question 615

A 14-year-old male being investigated for iron-deficiency anaemia is found to have numerous polyps in his jejunum. On examination he is also noted to have pigmented lesions on his palms and soles. What is the likely diagnosis? Hereditary non-polyposis colorectal carcinoma Gardner's syndrome Familial adenomatous polyposis Peutz-Jeghers syndrome Hereditary haemorrhagic telangiectasia

Answer 615

Hereditary haemorrhagic telangiectasia is associated with mucocutaneous lesions and iron-deficiency anaemia but intestinal polyps are not a feature

Question 616

Features of Peutz-Jeghers syndrome

Answer 616

Peutz-Jeghers syndrome is an autosomal dominant condition characterised by numerous hamartomatous polyps in the gastrointestinal tract. It is also associated with pigmented freckles on the lips, face, palms and soles. Around 50% of patients will have died from a gastrointestinal tract cancer by the age of 60 years. Genetics autosomal dominant responsible gene encodes serine threonine kinase LKB1 or STK11 Features hamartomatous polyps in GI tract (mainly small bowel) pigmented lesions on lips, oral mucosa, face, palms and soles intestinal obstruction e.g. intussusception gastrointestinal bleeding Management conservative unless complications develop

Question 617

Answer 617

Peutz-Jeghers syndrome

Question 618

You see a 6 week-old baby boy for his routine baby check and note a small, soft, umbilical hernia on examination. What should you do? Advise parents to tape a coin over the area Refer for surgery Refer for ultrasound Watch and wait Arrange emergency admission

Answer 618

Small umbilical hernias are common in babies and tend to resolve by 12 months of age. Parents should be reassured no treatment is usually required but to be aware of the signs of obstruction or strangulation such as vomiting, pain and being unable to push the hernia in - this is rare in infants. Advise the parents to present the child at around 2 years of age if the hernia is still present to arrange referral to a surgeon. Attempts to treat the hernia by strapping or taping things over the area are not helpful and can irritate the skin.

Question 619

Answer 619

Question 620

The mother of a 6-week-old baby girl born at 32 weeks gestation asks for advice about immunisation. What should happen regarding the first set of vaccines? Give first set of vaccinations at 3 months (i.e. delay for 1 month) Give DTaP/IPV/Hib at 2 months but not PCV Give first set of vaccinations at 4 months (i.e. correct for gestational age) Give first set of vaccinations as per normal timetable but within hospital environment Give as per normal timetable

Answer 620

Give as per normal timetable

Question 621

You are asked to by the Primary Care Trust to design a program to improve the health of infants in the local community. What is the most common cause of death of infants greater than one month but less than one year old? Accidents Congenital disorders Sudden infant death syndrome Cancer Infection

Answer 621

Sudden infant death syndrome After the age of 1 year accidents are the most common cause of death in children

Question 622

A 12-year-old female from Bulgaria presents to the surgery. She reports being unwell for the past 2 weeks. Initially she had a sore throat but she is now experiencing joint pains intermittently in her knees, hips and ankles. On examination there are some pink, ring shaped lesions on the trunk and occasional jerking movements of the face and hands. What is the most likely diagnosis? Lyme disease Infective endocarditis Polyarticular juvenile idiopathic arthritis Rheumatic fever Still's disease

Answer 622

Rheumatic fever

Question 623

Features of rheumatic fever

Answer 623

Rheumatic fever develops following an immunological reaction to recent (2-6 weeks ago) Streptococcus pyogenes infection. Diagnosis is based on evidence of recent streptococcal infection accompanied by: 2 major criteria 1 major with 2 minor criteria

Question 624

Answer 624

Erythema marginatum

Question 625

Rheumatic fever criteria JONES CAFE PAL

Answer 625

**Major** Joint involvement O- myocarditis Nodules, subcutaenous Erythema marginatum Sydenham's chorea **Minor** CRP increased Arthralgia Fever Elevated ESR Proloned PRI Anamnesis of rheumatism Leukocytosis

Question 626

Evidence of recent strep infection

Answer 626

ASOT \>200iU/mL Hx of scarlet fever Positive throat swab Increase in DNAse B titre

Question 627

You are reviewing a 9-month-old child with suspected bronchiolitis. Which one of the following features should make you consider other possible diagnoses? Fine inspiratory crackles Rhinitis Feeding difficulties Temperature of 39.7ºC Expiratory wheeze

Answer 627

A low-grade fever is typical in bronchiolitis. SIGN guidelines advise that the presence of high fever should make the clinician carefully consider other causes before making the diagnosis.

Question 628

After birth which of the following happens in the foetus? The foramen ovale opens allowing blood to circulate into the pulmonary artery Haemoglobin A is replaced by Haemoglobin F, which has a lower affinity for oxygen and may lead to physiological jaundice in the newborn The umbilical veins and arteries remain open for several days The ductus arteriosus closes The first few breaths force lung fluid into the fetal alveoli

Answer 628

After birth, the foramen ovale, ductus arteriosus and umbilical vessels close within a few hours. After a few days Haemoglobin F is replaced by Haemoglobin A, which has a lower affinity for oxygen and may lead to physiological jaundice in the newborn, due to the breakdown of fetal blood cells. The first few breaths force lung fluid out of the fetal alveoli.

Question 629

Foraemn ovale

Answer 629

This allows blood to shunt from the right atrium to left atrium, without having to pass through the lungs. At birth the lungs become functional and the pulmonary pressure decreases, resulting in a left atrial pressure which exceeds the right atrial pressure. This forces the septum primum septum secundum together, functionally closing the foramen ovale. The septa eventually fuse, leaving a remnant of the foramen ovale, called the fossa ovalis.

Question 630

Ductus arteriosus:

Answer 630

This is a vessel connecting the pulmonary artery to the aorta which allows blood from the right ventricle to bypass the non-functioning fetal lungs. After birth this closes to form the ligamentum arteriosum. The closure of the ductus arteriosus allowed blood to circulate into the pulmonary artery and become oxygenated. If the ductus arteriosus fails to close patients are left with a patent ductus arteriosus (PDA) which causes left-to-right shunting and can lead to pulmonary hypertension, heart failure and arrhythmias.

Question 631

Truncus arteriosus

Answer 631

Ascending aorta and pulmonary trunk The division of the truncus arteriosus is triggered by neural crest cell migration from the pharyngeal arches. Problems with the migration may lead to transposition of the great arteries or tetralogy of Fallot

Question 632

Bulbis cordis

Answer 632

Right ventricle and smooth parts of left ventricle

Question 633

Primitive atria

Answer 633

Trabeculated parts of the left and right atria

Question 634

Primitive ventricle

Answer 634

Majority of left ventricle

Question 635

Left horn of the sinus venous

Answer 635

Coronary sinus

Question 636

Right horn of the sinus venous

Answer 636

Smooth part of the right atrium

Question 637

Right common cardinal vein and right anterior cardinal vein

Answer 637

Superior vena cava

Question 638

Umbilical artery

Answer 638

Medial umbilical ligaments

Question 639

Umbilical vein

Answer 639

Ligamentum teres hepatis (inside falciform ligament)

Question 640

Ductus arteriosus

Answer 640

Ligamentum arteriosum

Question 641

Ductus venous

Answer 641

Ligamentum venosum

Question 642

Urachus

Answer 642

The urachus is a fibrous remnant of the allantois, a canal that drains the urinary bladder of the fetus that joins and runs within the umbilical cord.[1] The fibrous remnant lies in the space of Retzius, between the transversalis fascia anteriorly and the peritoneum posteriorly.

Question 643

Pre-school wheeze in children

Answer 643

Wheeze is extremely common in pre-school children, with an estimated 25% of children having an episode of wheeze before 18 months. Viral-induced wheeze is now one of the most common diagnoses made on paediatric wards. There is however ongoing debate regarding the classification of wheeze in this age group and the most appropriate management. Over recent years, led by the European Respiratory Society Task Force, the favoured classification for pre-school wheeze is to divide children into one of two groups; episodic viral wheeze: only wheezes when has a viral upper respiratory tract infection (URTI) and is symptom free inbetween episodes multiple trigger wheeze: as well as viral URTIs, other factors appear to trigger the wheeze such as exercise, allergens and cigarette smoke Episodic viral wheeze is not associated with an increased risk of asthma in later life although a proportion of children with multiple trigger wheeze will develop asthma.

Question 644

Mx of episodic viral wheeze

Answer 644

Episodic viral wheeze treatment is symptomatic only first-line is treatment with short acting beta 2 agonists (e.g. salbutamol) or anticholinergic via a spacer next step is intermittent leukotriene receptor antagonist (montelukast), intermittent inhaled corticosteroids, or both there is now thought to be little role for oral prednisolone in children who do not require hospital treatment

Question 645

Mx Multiple trigger wheeze

Answer 645

trial of either inhaled corticosteroids or a leukotriene receptor antagonist (montelukast), typically for 4-8 weeks

Question 646

An 18-month-old boy is brought to the GP by his mother as she is concerned about his breathing. Three days ago he started with fever, cough and rhinorrhoea. For the past 24 hours his mother reports that he has been 'wheezy'. On examination his temperature is 37.9ºC, heart rate 126/min, respiratory rate 42/min and a bilateral expiratory wheeze is noted. You prescribe a salbutamol inhaler along with a spacer. Two days later the mother represents noting the inhaler has made little difference to the wheeze. Clinical findings are similar, although his temperature today is 37.4ºC. What is the most appropriate next step in management? ## Footnote Inhaled long-acting beta agonist Oral prednisolone Add in regular ipratropium bromide Oral montelukast or inhaled corticosteroid Oral amoxicillin

Answer 646

This child is likely to have a viral-induced wheeze, also known as episodic viral wheeze. First-line treatment is short-acting bronchodilator therapy. If this is not successful then either oral montelukast or inhaled corticosteroids should be tried.

Question 647

What is the prevalence of atopic eczema in children? 1-2% 2-5% 15-20% 11-12% 5-10%

Answer 647

Eczema occurs in around 15-20% of children and is becoming more common. It typically presents before 6 months but clears in around 50% of children by 5 years of age and in 75% of children by 10 years of age

Question 648

Featues of eczma

Answer 648

in infants the face and trunk are often affected in younger children eczema often occurs on the extensor surfaces in older children a more typical distribution is seen, with flexor surfaces affected and the creases of the face and neck

Question 649

Mx of eczma

Answer 649

Avoid irritants Simple emollients: prescribe large amounts in a ratio with topcial steroids of 10:1. If a topical steroid is being used the emollient should be applied first followed by waiting at least 30 minutes before applying topical steroids. Topical steroids In severe cases wet wraps and oral ciclosporin may be used

Question 650

Characteristic features of congenital rubella infection

Answer 650

Sensorineural deafness Congenital cataracts Congenital heart disease: PDA Glaucoma Other features include: Growth retardation Hepatosplenomegaly Purpuric skin lesions Salt and pepper chorioretinitis Micropthalmia CP

Question 651

Answer 651

Salt and pepper chorioretinitis Rubella

Question 652

TORCH

Answer 652

Toxoplasmosis Other: syphillis, VZV, parvovirus B19 Rubella CMV Herpes

Question 653

Characteristic features of toxoplasmosis vertical transmission

Answer 653

Cerebral calcification Chorioretinitis Hydrocephalus Other features: Anaemia Hepatosplenomegaly CP

Question 654

Characteristic of vertical CMV infection

Answer 654

Growth retardation Purpuric skin lesions Others: Sensorineural deafness Encephalitis/seizures Pneumonitis Hepatosplenomegaly Anaemia Jaundice CP

Question 655

TOF

Answer 655

VSD RVH Right ventricular outflow tract obstruction Overriding aorta

Question 656

What is the most common cause of cyanotic congenital heart disease?

Answer 656

TOF \*however, at birth transposition of the great arteries is the more common lesion as patients with TOF generally present at around 1-2 months

Question 657

Answer 657

Question 658

What determines the clinical severity of TOF?

Answer 658

The severity of the RV outflow tract obstruction

Question 659

Features of TOF

Answer 659

Cyanosis Causes a right to left shunt Ejection systolic murmur due to PS Right sided aortic arch seen in 25% CXR shows boot shaped heart, ECG shows RVH

Question 660

Answer 660

Boot shaped heart TOF

Question 661

Mx of TOF

Answer 661

Surgical repair often undertaken in two parts. Cyanotic episodes may be helped by beta blockers to reduce infundibular spasm

Question 662

What is the most common cause of hypertension in children? Renal vascular disease Congenital adrenal hyperplasia Renal parenchymal disease Coarctation of the aorta Phaeochromocytoma

Answer 662

Renal parenchymal disease

Question 663

Causes of HTN in children

Answer 663

Renal parenchymal disease Renal vascular disease Coarctation of the aorta Phaeo CAH Essential or primary HTN

Question 664

It is December and you are the paediatric foundation doctor. A five month old baby is admitted through the paediatric observation unit with tachypnoea, tachycardia and fever. On examination there is evidence of increased work of breathing with sub costal and diaphragmatic recession. There is widespread wheeze. You discuss the patient with your senior and a diagnosis of bronchiolitis is established. Oxygen is started but it is not deemed appropriate to begin intravenous fluids at this time. Which investigation is important to conduct in the management of this patient? ## Footnote Glucose Full blood count Nasopharyngeal aspirate Urea and electrolytes Arterial blood gas

Answer 664

Nasopharyngeal aspirate are recommended during the winter months to ascertain which children are suffering with respiratory syncitial virus positive bronchiolitis. By diagnosing these patients it helps with ward management of patients, placing RSV negative patients on a ward and RSV positive patients in a side room. Urea and electrolytes would only be appropriate if the patient was on IV fluids.

Question 665

Features of benign rolandic epilepsy

Answer 665

Form of childhood epilepsy which typically occurs between 4 and 12 years Seizures characteristically occur at night Typicall partial: paraesthesia affecting face but secondary generalisation amy occur. Child is otherwise normal EEG characteristically shows centro-temporal spikse Px is excellent with seizures stopping by adolescence

Question 666

Charlie is a 7 month old baby boy who presents to you with poor weight gain (50th to 10th centile), on examination he has an erythematous, blanching rash over his abdomen, colicky abdominal pain and vomiting after feeds. He has been breast feeding with top ups of 'Aptamil' formula. What is the most likely diagnosis? Pyloric stenosis Eczema Infantile colic Cows' milk protein intolerance Reflux

Answer 666

The correct answer is cows' milk protein intolerance. The following clues in the history would suggest the diagnosis of cows' milk protein intolerance: Multi-system involvement 7 months would suggest the new introduction of top up feeds which correlates with the symptoms Faltering growth along with the multi-system involvement would suggest cows' milk protein intolerance Charlie is older than the classical age of presentation for pyloric stenosis (2 to 8 weeks very rare above 6 months) The presentation is unusual for eczema, infantile colic and reflux due to the multi-system involvement in the history making cows' milk protein intolerance more likely.

Question 667

A newborn is found to have a number of congenital abnormalities including an extra finger on each hand, a cleft palate and lip, microphthalmia and microcephaly. Which of the following chromosomes is most likely to be affected in this child? 9 12 13 18 21

Answer 667

Patau syndrome is a chromosomal abnormality resulting in an extra full copy of chromosome 13 (trisomy 13). Like many of the chromosomal defects, physical and mental disability is common, in this case key distinguishing features to separate Patau's from other trisomy disorders include polydactyly, cleft lips and palates, microcephaly and microphthalmia. Many children die before within a year of birth but those who survive will often go on to show intellectual and motor disability.

Question 668

What are the most common #s associated with child abuse?

Answer 668

Radial Humeral Femoral

Question 669

What are the common #s not associated with NAI

Answer 669

Distal radial Elbow Clavicular Tibial

Question 670

Triad in shaken baby syndrome

Answer 670

Retinal haemorrhages Subdural haematoma Encephalopathy Caused by the intentional shaking of a child.

Question 671

A 14-year-old boy is brought in by his mother who noticed her child had repeated episodes of slurred speech and gait abnormalities. On musculoskeletal examination, you notice muscle weakness, dysdiadochokinesis and spinal scoliosis. What is the mode of inheritance of this condition? X-linked recessive Autosomal dominant Point mutation X-linked dominant Autosomal recessive

Answer 671

Firedrich's ataxia AR

Question 672

Triad in HUS

Answer 672

Acute renal failure MAHA Thrombocytopenia

Question 673

Causes of HUS

Answer 673

Post-dysentry- classically E Coli 0157:H7 Tumours Pregnancy Ciclosporin, OCP SLE HIV

Question 674

Ix in HUS

Answer 674

FBC: anaemia, thrombocytopenia, fragmented blood film U&E: acute renal failure Stool culture

Question 675

Mx of HUS

Answer 675

Supportive: fluids, blood transfusions, dialysis if required No role for Abx. PLEX reserved for cases not associated with diarrhoea

Question 676

A 6-year-old boy is diagnosed as having nephrotic syndrome. A presumptive diagnosis of minimal change glomerulonephritis is made. What is the most appropriate treatment? Cyclophosphamide Albumin infusion Plasma exchange Renal biopsy followed by prednisolone Prednisolone

Answer 676

A renal biopsy is only indicated if response to steroids is poor

Question 677

A 29-week-old baby is born premature and shortly after birth experiences tachypnoea and tachycardia along with chest wall retractions. The paediatrician notes that the neonate has a blue discolouration of the skin and commences continuous positive airway pressure (CPAP) and intravenous fluids before explaining to the parents that the lungs lack surfactant, a compound that helps people breathe. Which of the following cells are responsible for surfactant production? Microfold cells Alveolar macrophage Type 1 pneumocytes Type 2 pneumocytes Paneth cells

Answer 677

Type 1 pneuomcytes are involved in the process of gas exchange between the alveoli and the blood and type 2 pneumocytes produce pulmonary surfactant.

Question 678

Features of TTN

Answer 678

Transient tachypnoea of the newborn (TTN) is the commonest cause of respiratory distress in the newborn period. It is caused by delayed resorption of fluid in the lungs It is more common following Caesarean sections, possibly due to the lung fluid not being 'squeezed out' during the passage through the birth canal Chest x-ray may show hyperinflation of the lungs and fluid in the horizontal fissure Supplementary oxygen may be required to maintain oxygen saturations. Transient tachypnoea of the newborn usually settles within 1-2 days

Question 679

What is the most appropriate way to confirm a diagnosis of pertussis? Blood cultures Sputum culture Per nasal swab Urine for serology Throat swab

Answer 679

Per nasal swab- may take weeks to come back

Question 680

Causes of microcephaly

Answer 680

Normal variation e.g. small child Familial e.g. parent with small head Congenital infection Perinatal brain injury e.g. hypxoci ischaemic encephalopathy Fetal alcohol syndrome Patau and other chromsomonal syndromes Craniosynostosis

Question 681

How can squints be classified?

Answer 681

By to where the eye deviates The nose: esotropria Temporally: exotropia Superiorly: hypertropia Inferiorly: hypotropia

Question 682

A mother brings her son in to surgery as she suspects he has a squint. She thinks his right eye is 'turned inwards'. You perform a cover test to gather further information. Which one of the following findings would be consistent with a right esotropia? On covering the left eye the right eye moves medially to take up fixation The cover test could not be used to identify this type of defect On covering the left eye the right eye moves laterally to take up fixation On covering the right eye the left eye moves laterally to take up fixation On covering the right eye the left eye moves medially to take up fixation

Answer 682

On covering the left eye in this example the right eye moves laterally from the nasal (esotropic) position to take up fixation.

Question 683

A neonate who was born prematurely at 35 weeks gestation is registered at the Practice. He was very well after delivery, without any notable complications such as respiratory problems. How should his routine childhood immunisations be given? Adjust schedule for gestational age Give according to chronological age Refer to the hospital to receive first immunisations Start immunisations at 3 months old Delay until weight reaches 3.5kg

Answer 683

Babies who were born prematurely should receive their routine vaccinations according to chronological age; there should be no correcting for gestational age. Babies who were born prior to 28 weeks gestation should receive their first set of immunisations at hospital due to risk of apnoea.

Question 684

A 14-year-old attends surgery. She was diagnosed with having migraines three years ago and requests advice about options for treating an acute attack. Which one of the following medications is it least suitable to recommend? Aspirin Paracetamol + prochlorperazine Paracetamol + codeine Ibuprofen Paracetamol

Answer 684

Avoid aspirin in children \< 16 years as risk of Reye's syndrome Aspirin should be avoided in children due to the risk of Reye's syndrome. Codeine would also be a poor choice as it has limited benefit in migraine.

Question 685

Mx of migraine: acute treatment

Answer 685

First line: combination therapy with an oral triptan and an NSAID or an oral triptan and paracetamol (for young people aged 12-17 consider a nasal triptan) If the above measures are not effective or not tolearted offer a non-oral preparation of metoclopramide (NB risk of acute dystonic reactions in young children) or prochlorperazine and consider adding a non-oral NSAID or triptan

Question 686

Mx of migraine: prophylaxis

Answer 686

Should be given if patients are expereincing 2 or more attacks per month NICE adivses either topiramate or propranolol according to persons preference. Propranolol should be used in women of child bearing age as topiramate may be teratogenic. If these measures fail, NICE recommends a course of up to 10 sessions of acupuncture or gabapentin. Ribloflavin may be affected For women with premenstrual migrain, frovatriptan or zolmitriptan can be used as a mini-prophylaxis

Question 687

Rules re 5-HT in mx of migraine

Answer 687

Agonists used in acute treatments Antagonists used in prophylaxis

Question 688

You are reviewing a 11-month-old baby with a viral upper respiratory tract infection. She is clinically well but at the end of the consultation her mother asks you about her development. You notice that she points and babbles 'mama' and 'dada' but has no other words. She is shy and cries when you try to examine her. There is an early pincer grip and she can roll from front to back but she cannot yet sit without support. How would you describe her development? Normal development Global developmental delay Isolated delay in gross motor skills Delay in speech + social skills, possibly early autism Isolated delay in fine motor skills

Answer 688

Most babies can sit without support at 7-8 months so this probably represents a delay in gross motor skills. If still present at 12 months she should be considered for referral to a paediatrician. The other development features are normal for her age.

Question 689

Rotavirus vaccination features

Answer 689

it is an oral, live attenuated vaccine 2 doses are required, the first at 2 months, the second at 3 months the first dose should not be given after 14 weeks + 6 days and the second dose cannot be given after 23 weeks + 6 days due to a theoretical risk of intussusception Other points the vaccine is around 85-90% effective and is predicted to decrease hospitalisation by 70% offers long-term protection against rotavirus

Question 690

Meera brings her 5 year old daughter Reena to the surgery who is being treated for acute lymphoblastic leukaemia (ALL) for review as Reena's classmate has been sent home from school with chickenpox. Reena is asymptomatic currently. Meera is unclear if Reena has suffered with chicken pox previously. What would be the correct management? Admit urgently Send home and come back if symptomatic Prescribe aciclovir Prescribe varicella zoster immunoglobulin Urgent bloods for varicella zoster antibodies

Answer 690

'People who have had a significant exposure to chickenpox and who are immunocompromised should be tested for varicella-zoster antibody, regardless of their history of chickenpox. Test for varicella-zoster immunoglobulin G (IgG) antibodies in primary care if test results can be available within 2 working days of first exposure. If this is not possible, urgently seek specialist advice because testing in secondary care and/or varicella-zoster immunoglobulin prophylaxis may be needed.'

Question 691

A newborn baby has their blood glucose measured on the post natal ward as part of the neonatal hypoglycaemia protocol due to low birthweight. It measures 2.9mmol/L. The midwife asks you what you want to do next? Admit to the Special Care Baby Unit (SCBU) for NG feeding Offer additional feed if willing Administer 100 mls intravenous 20% glucose Measure blood glucose again in three hours time Take blood sample for a formal glucose measurement

Answer 691

Neonatal hypoglycaemia is a common medical problem affecting neonates. This usually represents adaption to extrauterine life as opposed to any significant underlying medical problems. In the neonate blood glucose levels of \>2.5mmol/L are usually regarded as normal. Formal measurements may be needed to confirm readings of either extreme as they are more reliable. If measurements are consistently \>2.5mmol/L then monitoring can be stopped.

Question 692

Which one of the following statements regarding absence seizures is incorrect? Typical age of onset of 3-10 years old Sodium valproate and ethosuximide are first-line treatments Seizures may be provoked by a child holding their breath There is a good prognosis The EEG characteristically shows a bilateral, symmetrical 3Hz spike and wave pattern

Answer 692

Seizures are characteristically provoked by hyperventilation

Question 693

Features of fetal varicella sndrome

Answer 693

Rsik of FVS following amternal varicella exposure is 1% IF OCCURS BEFORE 20W. Skin scarring Eye defects: microphthalmia Limb hypoplasia Microcephaly LD

Question 694

Pregnant woman with VZV rash

Answer 694

oral aciclovir should be given if pregnant women with chickenpox present within 24 hours of onset of the rash

Question 695

1/2 alpha chains absent in alpha thalassaemia?

Answer 695

If 1 or 2 alpha chains are absent then the blood picture would be hypochromic and microcytic, but the Hb level would be typically normal

Question 696

Loss of 3 alpha chains in alpha thalassaemia?

Answer 696

Hypocrhomic, microcytic anaemia with splenomegaly. = HbH disease

Question 697

Loss of 4 alpha chains in alpha thalassaemia?

Answer 697

Death in utero: hydrops fetalis, Bart's hydrops

Question 698

Causes of visual problems in children

Answer 698

congenital: infection, cataracts prematurity - retinopathy of prematurity cerebral palsy optic atrophy e.g. hydrocephalus, optic nerve hypoplasia albinism

Question 699

The UK has recently switched to the new growth charts based on the WHO growth standard for children under the age of 5 years. The new UK-WHO charts have a separate preterm section and a 0-1 year section. Key points

Answer 699

Key points ## Footnote based on data from breast fed infants and all ethnic groups the data matches UK children well for height and length but after 6 months UK children and slightly more heavy and more likely to be above the 98% centile preterm infants born at 32-36 weeks have a separate chart until 2 weeks post-term

Question 700

What is the pathophysiology of Fragile X?

Answer 700

Trinucleotide repeat disorder

Question 701

Features of fragile x

Answer 701

In males: LD Large low set ears, long thin face, high arched palate Macroorchidism Hypotonia Autism Mitral valve prolapse Features in females: range from normal to mild

Question 702

A 4-year-old boy is admitted after developing a haemarthrosis in his right knee whilst playing in the garden. The following blood results are obtained: Platelets220 \* 109/l PT12 secs APTT78 secs Factor VIIIc activityNormal What is the most likely diagnosis? Antithrombin III deficiency Von Willebrand's disease Antiphospholipid syndrome Haemophilia A Haemophilia B

Answer 702

A grossly elevated APTT may be caused by heparin therapy, haemophilia or antiphospholipid syndrome. A normal factor VIIIc activity points to a diagnosis of haemophilia B (lack of factor IX). Antiphospholipid syndrome is a prothrombotic condition

Question 703

6-week-old infant is referred from the health visitor due to failure to thrive. The infant has fallen from the 50th to 9th centile on growth chart for weight. On further questioning, the parents reveal the infant vomits following each meal, which have on occasions 'hit the wall.' The mother's pregnancy was unremarkable, with normal antenatal scans, and the infant was born by an uncomplicated vaginal delivery. There were no abnormal features noted at the newborn baby examination. What is the most likely diagnosis? Intussusception Infantile colic Cow's milk protein intolerance Galactosaemia Pyloric stenosis

Answer 703

In this question the most likely diagnosis is pyloric stenosis. Pyloric stenosis typically presents around 2-6 weeks of age. Infants tend to have projectile vomiting following feeds and remain hungry after vomiting. There may be an olive shaped mass in the right upper quadrant due to hypertrophy of the pylorus, and 'waves of peristalsis' may be seen following a test feed.

Question 704

Which one of the following statements regarding infantile spasms is incorrect? EEG shows hypsarrhythmia in the majority of children Carries a good prognosis More common in male children Typically presents in the first 4 to 8 months Causes characteristic 'salaam' attacks

Answer 704

Carries a good prognosis

Question 705

West Syndrome

Answer 705

Infantile spasms

Question 706

Features of infantile spasms

Answer 706

Type of childhood epilepsy which typically presents in the first 4-8m of life and is more common in males. Often assocaited with serious underlying condition and the prognosis is poor. Characteristics salaam attacks: flexion of the head, trunk and arms followed by extension of the arms. This lasts 1-2 second but may be repeated up to 50 times Progressive mental handicap

Question 707

Ix in West Syndrome

Answer 707

EEG demonstrates hypsarrhythmia in 2/3rds of infants CT demonstrates diffuse or localised brain disease in 70% e.g. tuberous sclerosis

Question 708

Answer 708

Hypsarrhythmia West Syndrome

Question 709

Mx of West Syndrome

Answer 709

Poor Px Vigabatrin is now considered first-line therapy ACTH is also used

Question 710

Features of Bartter's syndrome

Answer 710

Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl- cotransporter in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine causes of hypokalaemia such as Conn's, Cushing's and Liddle's syndrome which are associated with hypertension) Features usually presents in childhood, e.g. Failure to thrive polyuria, polydipsia hypokalaemia normotension weakness

Question 711

What are the primitive reflexes?

Answer 711

Moro Grasp Rooting Stepping

Question 712

Moro reflex

Answer 712

Head extension causes abduction followd by adduction of the arms Present from birth to 3-4m old

Question 713

Grasp reflex

Answer 713

Flexion of fingers when object placed in palm Present from birth to around 4-5 months of age

Question 714

Rooting reflex

Answer 714

Assists in breastfeeding Present from birth to 4m of age

Question 715

Stepping reflex

Answer 715

AKA walking reflex Present from birth to around 2 onths of age

Question 716

What are the 4 main Sickle cell crises?

Answer 716

Thrombotic 'painful' crises Sequestration Aplastic Haemolytic

Question 717

What are the features of thrombotic crises in SCD

Answer 717

AKA painful crises or vaso-occlusive crises Precipitated by infection, dehydration, deoxygenation Infarcts occur in various organs including the bones e.g. AVN of the hip, hand-foot syndrome (dactylitis) in children, lungs, spleen and brain

Question 718

Sequestration crises in SCD

Answer 718

Sickling within the organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia Acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low pO2 The most common cause of death after childhood

Question 719

In the context of an African patient suffering from a long-standing anaemia

Answer 719

Acute chest syndrome Multiple pulmonary infiltrates

Question 720

Aplastic crises in SCD

Answer 720

Caused by infection with parvovirus Sudden fall in Hb

Question 721

Hamolytic crises in SCD

Answer 721

Rare Fall in Hb due to an increased rate of haemolysis

Question 722

A 60 year-old man with haemophilia A has just become a grandfather. He wants to know what the chances are of his daughter's son having haemophilia. Her daughter's partner is well with no past medical history. What is the probability that his daughter's son has haemophilia A? Impossible to calculate 50% 25% No increased risk 100%

Answer 722

Haemophilia A is an X-linked recessive disease. This means that all female offspring of affected men will be carriers. There is then a 50% chance of these females passing the gene on. If the female's children are male, they will therefore have a 50% chance of having the condition.

Question 723

A man brings his 18 month old daughter to your GP clinic. She has had coryzal symptoms for the last 2 days. Last night, she started with a barking cough and a mild temperature of 37.8º. On examination, there is a mild stridor when mobilising, with no recessions visible. Chest sounds clear with good air entry bilaterally. Temperature today remains at 37.8º, but all other observations are normal. What is the appropriate management? Admit to hospital Give nebulised adrenaline Give a stat dose of dexamethasone 150 micrograms/kg PO Give a salbutamol inhaler Start antibiotics

Answer 723

This is a child who has croup. This is an illness that usually starts with coryzal symptoms, and the child then develops a seal like, barking cough. The first stage is to work out how serious a case of croup this child has. Generally recommendations include: Mild croup: Occasional barking cough with no stridor at rest No or mild recessions Well looking child Moderate croup: Frequent barking cough and stridor at rest Recessions at rest No distress Severe croup: Prominent inspiratory stridor at rest Marked recessions Distress, agitation or lethargy Tachycardia In this case, the child would have mild croup. Admission to hospital is only considered for moderate or severe croup, or if an alternative severe diagnosis like epiglottitis is suspected. It would not be appropriate in this case. Nebulised adrenaline would only be used for children who were distressed, or who had a severe stridor. It would be not be used in this case as this child is well at rest with only a mild stridor on movement. A salbutamol inhaler would only help if the child had wheeze, which she does not in this case. It would not give her any benefit. Antibiotics are not indicated in croup as it is a viral illness. Systematic reviews have shown that steroids can ease symptoms within a few hours. They also lead to fewer reattendances and fewer hospital admissions. Mild croup will resolve on its own, but Dexamethasone has been shown to be of some benefit.

Question 724

Mx of CF

Answer 724

Regular (\>BD) chest PT and psotural drainage. Deep breathing exercises are also useful High calorie diet including high fat intake Vitamin supplementation Pancreatic enzyme supplements taken with meals Heart and lung transplant

Question 725

What is the most common cause of hypothyroidism in children in the UK?

Answer 725

Autoimmune thyroiditis Other causes include: Post total-body irradiation (i.e. in a child previously treated for ALL) Iodine deficiency (most common cause in the developing world)

Question 726

A 15-year-old collapses and dies whilst playing football and school. He had no past medical history of note. Post-mortem examination reveals asymmetric hypertrophy of the interventricular septum. Given the likely diagnosis, what is the chance his sister will also have the same underlying disorder? 0% 25% 50% 100% 66%

Answer 726

The underlying diagnosis is hypertrophic obstructive cardiomyopathy which is an autosomal dominant disorder. His sister therefore has a 50% chance of being affected.

Question 727

Def: HOCM

Answer 727

AD disorder of muscle tissue caused by defects in te genes endocing contractile proteins. Most common defects involves a mutation in the gene encoding beta-myosin heavy chain protein or myosin binding protein C

Question 728

Features of HOCM

Answer 728

Often asymptomatic Dyspnoea, angina, syncope Sudden death (most commonly due to ventricular arrythmias), arrythmias, HF Jerky pulse, large a waves, double apex beat ESM

Question 729

Jerky pulse, large a waves, double apex beat

Answer 729

HOCM

Question 730

Conditions associated with HOCM

Answer 730

Friedreich's ataxia WPW

Question 731

Echo findings in HOCM MR SAM ASH

Answer 731

Mital regurgitation Systolic anterior motion (SAM) of te anterior mitral valve leaflet) Asymmetric hypertrophy (ASH)

Question 732

ECG findings in HOCM

Answer 732

LV hypertrophy Progressive T wave inversion Deep Q waves AF may be seen occasionally

Question 733

Fever + Symptoms and signs: of meningococcal disease

Answer 733

Non-blanching rash, paritculalry in conjunction with: an ill looking child Lesions larger than 2mm (=purpura) CRT \>3s Neck stiffness

Question 734

Fever + Symptoms and signs: of meningitis

Answer 734

Neck stiffness Bulging fontanelle Decreased level of conciousness Convulsive status epilepticus

Question 735

Fever + Symptoms and signs: Herpes simplex encephalitis

Answer 735

Focal neurological signs Focal seizures Altered levels of consciousness

Question 736

Fever + Symptoms and signs: pneumonia

Answer 736

Tachypnoea Crackles in the chest Nasal flaring Chest indrawing Cyanosis SaO2 \<95%

Question 737

Fever + Symptoms and signs: UTI

Answer 737

Vomiting Poor feeding Lethargy Irritability Abdominal pain or tenderness Urinary frequency or dysuria Offensive urine/haematuria

Question 738

Fever + Symptoms and signs: septic arthritis/osteomyelitis

Answer 738

Swelling of limb or joint Not using an extremity Not weight bearing

Question 739

Fever + Symptoms and signs: Kawasaki

Answer 739

Fever lasting \>5d and at least 4 from: bilaterla conjuncitval injection change in URT mucous membranes Change in the peripheral extremitis Polymorphous rash Cervical lymphadenopathy

Question 740

What are the most common causes of pharyngitis?

Answer 740

Adenovirus, enterovirus, rhinovirus In older children Group A beta haemolytic strep

Question 741

Def: tonsilitis

Answer 741

Form of pharyngitis where there is intensive inflammation of the tonsils, often with a purulent exudate

Question 742

Common pathogens causing tonsilits?

Answer 742

Group A beta haemolytic strep EBV

Question 743

What differentiates between EBV and GAS tonsilitis

Answer 743

EBV surface exudate is more membranous, group a strep commonly gives a constitutional disturbance and has a white tonsilar exudate

Question 744

Answer 744

EBV tonsiltiis

Question 745

Answer 745

Group A strep tonsilitis

Question 746

Mx of tonsilitis

Answer 746

Do not examine if ?acute epiglottitis Assess airway and ability to feed Abx commonly given, often penicllin or erythromycin if pen allergic Analgesic with ibuprofen NB: avoid amoxicillin as maculopapular rash may develop secondary to EBV. This is to eradicate organism: 10d of antibiotics Admit if unable to swallow solids/liquids.

Question 747

Centor score components

Answer 747

\<14 or \>45 = +1 Exudate or swelling on tonsils Tender/swollen anterior cervical lymph nodes Fever \>38 Cough absent 2 or 3: throat culture and treat with antibiotic 4-5 points: treat empirically with an antibioitc -risk of strep infection 56%

Question 748

Indications for tonsillectomy

Answer 748

Useful in children with recurrent tonsilitis Recurrent severe tonsilltiis A peritonsillar abscess Obstructive sleep apnoea

Question 749

Answer 749

Quinsy abscess

Question 750

Indications for adenoidectomy

Answer 750

Grow faster than airway between 2-8y of age Can cause narrowing of the airway lumen Recurrent otitis media with effusion and hearing loss Obstructive sleep apnoea (absolute indication)

Question 751

Symptoms of HSV

Answer 751

Asymptomatic Gingiovstomatitis: most common manifestation of the virus in children, painful vesciles on the muth, hard palate, lips and tongue Herpetic eczema Herpetic whitlows: oedmatous white pusttules on the site of broken skin on the fingers Eye infection Meningitis/encephalitis Penumonia and disseminated infeciton in the immunocompromised

Question 752

Cx of EBV

Answer 752

Swelling of the pharynx so that is causes airway obstruction and difficulties feeding

Question 753

Symptoms of EBV infection

Answer 753

Fever Malaise Tonsilopharyngitis limiting oral intake Cervical lymphadenoathpy Hepatosplenomegaly Maculopapular rash Jaundice Petechiae on the soft palate

Question 754

Ix in EBV

Answer 754

Atypical lymphocytes Monospot test Abs vs EBV

Question 755

Mx of EBV

Answer 755

Supportive Steroids if airway is compromsed 5% grow strep so treat with penicllin (not ampicillin or amoxicillin as these will cause florid maculopapular rash)

Question 756

Erythema infantiosum

Answer 756

Fever Malaise Myalgia Slapped cheek Complications: arthrtis, arthralgia, aplastic anaemia Causes fetal hydrops in utero Caused by parvovirus B19

Question 757

Answer 757

Erythema infectiosum (slapped cheek)

Question 758

Coxsackie virus causes?

Answer 758

Hand, foot and mouth disease

Question 759

Features of hand foot and mouth

Answer 759

Painful vesciular lesions on the hands, feet, mouth and tongue and often on the buttocks Mild systemic features Subsides within a few days with fluids and analgesia

Question 760

Bornholms Disease

Answer 760

Bornholm disease or epidemic pleurodynia or epidemic myalgia[1] is a disease caused by the Coxsackie B virus or other viruses.[2] Pleuritic chest pain, fever, myalgia- resolves within a few days

Question 761

Draw the clinical features of chickenpox

Answer 761

Question 762

What is the typical rash in chickenpox?

Answer 762

200-500 lesions start on head and trunk progressing to peripheries. Appear as crops of papules, vesicles with surrounding erythema and pustules at different times Itching and scratching may cause scarring. If new lesions appear beyond 10d suggestive of defective cellular immunity

Question 763

What are the cx of chickenpox

Answer 763

Bacterial superinfection: Staph, strep May lead to toxic shock syndrome or necrotising fasciitis CNS: Cerebellitis Generalised encephalitis Aseptic meningitis Immunocompromised: Haemorrhagic lesions Pneumonitis Progressive and disseminated infection DIC

Question 764

Rash in measles

Answer 764

Starts behind the ears, spreads downwards to the whole of the body Discrete maculopapular rash intitially whch becomes blotchy and confluent May desquamate

Question 765

Draw the clinical course of measles

Answer 765

Question 766

What are the symptoms of mumps

Answer 766

Fever Malaise Parotitis: pain on chewing or swalling Transient unilateral heaing loss

Question 767

Cx of mumps

Answer 767

Meningitis/encephalitis Orchitis Pancreatitis

Question 768

Symptoms of rubella

Answer 768

Low grade fever Maculopapular rash (non itch unlike adults) Post auricular lymphadenopathy

Question 769

Mx of Lyme disease

Answer 769

\>12y: doxy \<12: amoxicillin Neuro or cardio: IV ceftraixone

Question 770

Treatment of impetigo

Answer 770

Topical abx e.g. mupirocin More severe infections: fluclox or co-amoxiclav (as children prefer the taste) Nasal carriage can be eradicated with a nasal cream containing mupirocin or chlorhexidine and neomycin

Question 771

Aetiology of peri-orbital cellulitis?

Answer 771

Infants: Staph or strep Hib in unvaccinatied or trauma Older: dental abscess or paranasal sinus infection

Question 772

Symptoms of peri-orbital cellulitis

Answer 772

Tenderness, oedema of the eyelid, erythema and fever

Question 773

Cx of periorbital cellulitis

Answer 773

Orbital cellulitis: if left untreated, can get pain on ocular movementm, proptosis, decreased visual actuity Can progress to abscess formation, meningitis and cavernous sinus thrombosis

Question 774

Ix in peri-orbital cellultis?

Answer 774

CT to exclude posterior spread LP to exclude menignits

Question 775

Mx of peri-orbital cellulitis

Answer 775

IV Abx to prevent posterior spread

Question 776

Answer 776

Peri-orbital cellultitis

Question 777

SCALDED SKIN SYNDROME

Answer 777

Infection and separation of the epidermal to granular layers due to an exfoliative staph toxin. Rare. fever and malaise a purulent, crusting, localised infection around the eyes, nose and mouth with subsequent widespread erythema and tenderness of the skin. Areas of epidermis separate on gentle pressure (Nikolsky sign), leaving denuded areas of skin which subsequently dry and heal without scarring.

Question 778

Mx of scalded skin syndrome

Answer 778

IV Abx Analgesia Fluid maintenance

Question 779

Answer 779

Scalded skin syndrome

Question 780

Necrotising fascititis

Answer 780

Severe skin infection extending from the dermis\_\> fascia-\> muscle Caused by Staph or Group A strep +/- anaerobic bacteria Systemically unwell Severe pain Necrotic centre with damaged tissue

Question 781

Mx of necrotising fascititis

Answer 781

IV Abx Surgical debridement Possible ICU admission

Question 782

Answer 782

Necrotising fasciitis

Question 783

Pathophysiology of bacterial meningitis

Answer 783

Infection of the meninges usually follow bacteraemia Inflammation and endothelial damage à cerebral oedema à raised ICP à decreased blood flow à CEREBRAL CORTICAL INFARCTION Fibrin deposits block CSF resorption by the arachnoid villi à hydrocephalus

Question 784

Meningitis in the Neonate-3m

Answer 784

GBS E Coli and other coliforms lIsteria

Question 785

Meningitis in 1m-6y

Answer 785

N meningitidies Strep penumoniae HiB

Question 786

Meningitis \>6y

Answer 786

Neisseria meningitidis Strep penumoniae

Question 787

Cx of meningitis

Answer 787

Hearing loss Local vasculitis Local cerebral infarctaion Subdural effusion Hydrocephalus Cerebral abscess

Question 788

Signs of raised ICP in child

Answer 788

Reduce conscious level Abnormal papillary response Abnormal posturing

Question 789

Kernig's sign

Answer 789

With the child lying supine and with hips and kness flexed, there is back pain on extension of the knee

Question 790

Cushing's triad

Answer 790

Bradycardia HTN Abnormal pattern of breathing = Raised ICP

Question 791

Ix in meningitis

Answer 791

FBC and differential count Blood glucose and blood gas CRP and coag screen U&Es LFTs MCS blood, throat swab, urine, stool RAAT for meningitis organisms (blood, CSF, urine) LP (CSF)

Question 792

Mx of meningitis

Answer 792

Abx: third generation cephalosporin: cefotaxime or ceftriaxone Dexamethasoone if beyond neonatal period to minimise risk of LT Cxs Supportive Prophylaxis with rifampicin

Question 793

What are the contraindications to LP

Answer 793

Cardiorespiratory instability Focal neurological signs Signs of raised ICP Coagulopathy Thrombocytopenia Local infection at site of LP If it causes undue delay in starting antibiotics

Question 794

Brudzinski sign

Answer 794

Flexion of the neck with the child supine causes flexion of the knees and hips

Question 795

Aetiology of encephalitis

Answer 795

Direct invasion of the cerebrum by a neurotoxic virus (e.g. HSV) Delayed brain swelling following a disordered neuroimmunological response to an antigen, usually a virus (post-infectious encephalopathy) e.g. following chickenpox A slow virus infection, e.g. HIV or subacute sclerosing panencephalitis (SSPE) following measles Enteroviruses, respiratory viruses and herpes virus (UK) Mycoplasma, Borrelia burgdoferi, Bartonella henselae, rickettsial infections and the arboviruses (worldwide)

Question 796

Ix in encephalitis

Answer 796

EEG and CT/MRI PCR of CSF

Question 797

Mx of encephalitis

Answer 797

High dose IV aciclovir even if HSV not confirmed Proven cases should be treated with 3 weeks aciclovir as relapses can occur

Question 798

What are the tropical causes of fever in a returning child

Answer 798

Malaria Typhoid Dengue Gastroenteritis and dysentry Viral haemorrhagic fevers

Question 799

Cx of malaria

Answer 799

Severe anaemia Cerebral malaria

Question 800

Symptoms of malaria

Answer 800

Typically the onset is 7-10d after innoculation Paroxysms of fever, shaking chills and sweats (every 48-72h) Non-specific symptoms: headache, cough, fatigue, malaise, shaking chills, arthralgia, myalgia, D+V, nausea, lethargy jaundice

Question 801

Ix in malaria

Answer 801

FBC clotting profile U&Es LFTs Blood glucose and blood gas Urinalysis Giemsa-stained thick and thin blood film (thick confirms Dx, thin-spp)

Question 802

Anti-malarial chemoprophylaxis

Answer 802

Quinine

Question 803

Rx in malaria

Answer 803

Quinine-based Rx for P falciparum Chloroquine for other forms

Question 804

Cx of typhoid

Answer 804

GI perforation Myocarditis, hepatitis, nephritis

Question 805

Symptoms of typhoid

Answer 805

Worsening fever, dull frontal headaches, cough and abdominal pain, anorexia, malaise and myalgia GI symptoms may not appear until the second week Splenomegaly, bradycardia and rose-coloured spots on the trunk

Question 806

Rx of typhoid

Answer 806

Cotrimoxazole, chloramphenicol or ampicillin Multi-drug resistant: 3rd generation cephalosporin or azithromycin

Question 807

Symtpoms of denge fever

Answer 807

Fine erythematous rash, high fever, headaches, arthralgia and myalgia, vomiting, haemorrhagic signs, lethargy Hepatomegaly, abdominal distension Severe forms: leukopenia, severe thrombocytopenia, haemorrhage, plasma leakage

Question 808

Dengue haemorrhagic fever/Dengue shock syndrome

Answer 808

Previously infected child has a subsequent infection with a different strain of the virus Severe capillary leak syndrome à hypotension & haemorrhagic manifestations

Question 809

Ix in Kawasaki

Answer 809

Clinical evaluation Inflammatory markers: CRP, ESR, WBC Plt Echo at 6w to confirm absence of aneurysm No specific diagnostic test

Question 810

Rx in Kawasaki

Answer 810

\<10d: IVIG Aspirin to reduce the risk at high anti-inflammatory dose Second dose of IVIG if fever recurs Persistent inflammation and fever: Infliximab, steroids or ciclosporin GCAA: LT warfarin therapy with close cardiology follow up

Question 811

Mx of septicaemia

Answer 811

May require transfer to ICU ABC Airaway clearance and ventilation should correct any acidosis Septic screen Fluids Montior CVP to assess fluid balance and urine output Cardiogenic dysfunction: inotropic support DIC: FFP, platelet transfsion Abx

Question 812

Sepsis 6

Answer 812

Deliver high-flow oxygen. Take blood cultures. Administer empiric intravenous antibiotics. Measure serum lactate and send full blood count. Start intravenous fluid resuscitation. Commence accurate urine output measurement.

Question 813

Causes of bronchiolotisis?

Answer 813

RSV (80%) Human metapneumoviur parainfluenza rhinovirus adenovirus

Question 814

Bronchiolotis \>1y?

Answer 814

Rare (0% are in 1-9m

Question 815

Risk factors for severe bronchiolitis?

Answer 815

Prem with bronchopulmonary dysplasia/underlying lung disease/ congenitla heart disease

Question 816

Cxs of bronchiolitis?

Answer 816

Permanent damage to ariways-\> bronchiolitis obliterans (adenovirus) Recrreunt cough and wheeze

Question 817

What is the aetiological agent in bronchiolitis obliternas?

Answer 817

Adenovirus

Question 818

Ix in bronchiolitis?

Answer 818

Pulse oximery Blood gas (in severe disease): hypercarbia indication for ventilatory support CXR rarely helpful NPA PCR is goldstandard for Dx

Question 819

Mx of bronchiolitis

Answer 819

Supportive Infection control Humidified O2 via nasal cannulae +/- ventilation: CPAP, full Infection control measures

Question 820

Palivizumab

Answer 820

Preventative mAb vs RSV used in high risk prems

Question 821

Answer 821

Bronchiolitis

Question 822

Answer 822

Question 823

Def: croup

Answer 823

Laryngoracheobronchitis Muscoal inflammation and increased secretions affecting the airway Oedema of the subglottic area-\> tracheal narrowing

Question 824

What year is croup most common?

Answer 824

Second year of life (6m to 6y) Autumn

Question 825

Causes of croup

Answer 825

95% viral: parainfluenza human metapneumovirus RSV IFV

Question 826

Mx of croup

Answer 826

Most resolves spontaneously Steroids: oral dexamethaonse/prednisolong, nebulised budenoside Admit in severe illness, \<1y, signs of dehydration Nebulised epinephrine via facemask with anaesthetic input due to risk of rebound Tracheal intubation

Question 827

Features of acute epiglottitis

Answer 827

Occurs over hours No preceing croyza Absent cough Unable to drink Drooling slaiva Very ill \>38.5 deg temperature Soft, whispering striodr Muffled voice EMERGENCY

Question 828

Cause of pneumonia in newborn?

Answer 828

Organisms from maternal genital tract e.g. GBS, gram \_ve enterococci

Question 829

Cause of pneumonia in infants?

Answer 829

Mainly viral: RSV also pneumococcus, H infleunza Infrequently but serious Staph

Question 830

Causes of pneumonia in \>5y

Answer 830

Mainly bacterial M pneumoniae Pneumococcus Chlamydia penumoniae

Question 831

Cx of pneumonia

Answer 831

Pleural effusion Emphyema Fibrin strands Septations

Question 832

Symptoms of penuemonia

Answer 832

Preceded by URTI-\> fever, difficulty breathing Cough, lethargy, poor feeding, localised chest/abdominal pain (suggestitve of bacterial) Tachypnoeaic with nasal falring, chest indrawing classic consolidation signs

Question 833

Ix of pnuemonia

Answer 833

NPA FBC CRP ESR CXR (not rountiely done)

Question 834

Indications for admission in pneumonia?

Answer 834

If O2 \<93, severe tachpynoea, SOB, grunting, apnoea, not feeding, family unable to support

Question 835

Mx of pneumonia

Answer 835

Supportive: O2, analgesia, IV fluids Abx: Newborns: broadspectrum IV abx Older infants: oral amoxicillin Complicated/unresponsive- co-amoxiclav \>5 years: amoxicillin or ertythromycin Drainage of empyema with a a chest drain +/- urokinase (fibrinolytic)

Question 836

Chest examination in CF and Ix

Answer 836

Hyperinflated chest Coarse inspiratory crackles/expiratory wheeze Clubbing Low elastase in faees suggestive of pancreatic insufficiency LFT FBC CXR if infection suspected.

Question 837

Dx of CF

Answer 837

Heel prick testing Sweat test Confrimation through genotyping

Question 838

CF sweat test

Answer 838

Sweat stimulated by pilocarpine Sweat collected in capillary tube or special filter paper Measures concentration of chloride in sweat

Question 839

Cl cut offs in CF

Answer 839

60-125= CF 10-40= normal

Question 840

Treatment of uncomplicated meconium ileus

Answer 840

Gastrografin enema My require surgery

Question 841

Respiratory mx of CF

Answer 841

Regular spirometry BD PT Continuous oral abx- fluclox as a prophylactic Azithromycin can also be given dialy Infections due to P aeuriginosa can cause rapid deterioriation- daily nebulised antipseudomonal Neublised DNAse and hypertonic saline can be used to decrease viscosity Bilateral luing transplant is the only treatment for end stage CF

Question 842

Nutritional Mx of CF

Answer 842

Pancreatic insufficeincy treateed with enteric coated pancreatic replacement therapy taken with all meals and snacks High calorie, high fat diet Regular ursodeoxycholic acid improveds bile flow Intestinal obstructions can be cleared by gastrografin

Question 843

MDT of CF

Answer 843

PT Dieticias Teachers Primary care team Specialist nurse

Question 844

Non-respiratory problems of CF

Answer 844

DM Delayed puberty Biliary atresia Male infertility

Question 845

Hx in CF

Answer 845

Chronic cough +/- wheezing Frequent chest infections FTT Frequent, bulky, greay stools History of meconium ileus FHx of CF

Question 846

Def: laryngomalacia

Answer 846

Congenital cause of upper airway obstruction

Question 847

Aetiology of laryngomalacia

Answer 847

Most common congential laryngeal abnromality is where the larynx is soft and floppy and collapses during breathing due to abnormalities of the laryngeal cartilage

Question 848

Cx of laryngomalacia

Answer 848

Associated with GORD Resp distress and FTT is rare 20% have another airway abnormality

Question 849

Symptoms of laryngomalacia

Answer 849

Inspiratory stridoer in an otherwise well child in the first few weeks of life Stridor exacerbated by: crying feeding lying supin intercurrent chest infection

Question 850

DDx for pneumonia

Answer 850

URTI Bronchiolitis Asthma Non-specific viral infection Inhaled foreing body

Question 851

What differentiates between viral and bacterial aetiology on a CXR in pneumonia

Answer 851

Bacterial is focal Viral may be difssue

Question 852

Features of mycoplasma pneumonia

Answer 852

Insidious onset Cold agglutinins

Question 853

DDx for laryngomalacia

Answer 853

Laryngeal web Laryngeal atresia

Question 854

Gold standard Dx for laryngomalacia

Answer 854

Flexible laryngoxcopy reveals an omega shaped epiglottis and prolapse over the larynx during inspiration

Question 855

Mx of laryngomalacia

Answer 855

Strioder may worsen in first 6m but usually resolves after 2y O2 sats monitored Watch and wait approach with treatment of concurrent infection or GORD Sx for severe cases: FTT, cor pulmonale, obstructive sleep apnoea

Question 856

What is the most common chronic respiratory disorder of childhood?

Answer 856

Heart failure

Question 857

Causes of heart failure in newborn

Answer 857

Due to obstructed systemic circulation: Hypoplastic left heart syndrome Crticial aortic valve stenosis Severe coarctation of the aorta Interruption of arotic arch

Question 858

Causes of heart failure in infants

Answer 858

Due to high pulmonary blood flow: VSD AVSD Large PDA

Question 859

Causes of heart failure in older children

Answer 859

Rheumatic heart disease Cardiomyopathy Eisenmenger syndrome

Question 860

Eisenmenger syndrome

Answer 860

Irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow

Question 861

Risk factors for heart failure in children

Answer 861

Familial Intrauterine e.g. rubella Drugs: Li and ETOH Maternal DM Maternal PKU Prem

Question 862

Symptoms of heart failure

Answer 862

Breathlessness Poor feeding Recurrent chest pain Sweating Cardiomegaly Gallop rhythm Tachycardia Hepatomegaly Cool peripheries

Question 863

Ix in HF

Answer 863

Clinical Neonatal murmurs Blood gases Full infection to exclude other causes of CV collapse Echo is gold standard Cardiac catheterisation may be necessary for more severe cases to assess the extent of the problem

Question 864

Conditions to consider in infant with tachypnoea or wheeze

Answer 864

Bronchiolitis Pneumonia Trnaisnt early wheezing Non-atpopic wheezing Atopic asthma Cardiac failure Inhaled foreign body Aspiration of feed

Question 865

Causes of URT obstruction

Answer 865

Croup Epiglottis Bacterial tracheitis Smoke inhalation Trauma Retropharyngeal abscess Laryngeal foreign body Allergic larygneal angioedema (seen in anaphylaxis and recurrent croup) Hypocalcaemia EBV causing severe cervical node swelling Measles Diptheria

Question 866

Which age group most at risk of forgein body aspiration

Answer 866

Toddlers

Question 867

Where is foreign body most commonly found

Answer 867

Right main bronchus

Question 868

Mx of inahled forgein body?

Answer 868

Heimlich Bronschoscopy

Question 869

Features of bacterial tracheitis

Answer 869

High fever, toxic Loud, harsh stridor

Question 870

Conditions to consider in child with stridor

Answer 870

Croup Epiglottitis Bacterial tracheitis Inhaled foreing body Laryngomalacia

Question 871

Aetiology of asthma

Answer 871

Bronchial inflammation Bronchial hyperresposiveness Airway narrowing

Question 872

Risk facors for asthma

Answer 872

Genetic predisposition Atopy: eczema, rhinocojunctivitis, food allergy Environmental triggers: URIT, allergens, smoking, cold air, exercise, anxiety

Question 873

Key features of asthma

Answer 873

Wheeze: polyphonic on more than one occasion Cough, breathlessness, chest tightness Worse at night/early morning Triggered e.g. exercise, pets, dust Interval (symptoms occur between acute exacerbations) Positive Fhx Positive response to asthma therapy

Question 874

O/E asthma (longstanding

Answer 874

Hyperinflation Generalised polyphonic expiratory wheeze Prolonged expiratory phase Harrison sulci Examin skin: eczma, nasal mucosa and growth (impaired growth in severe asthma)

Question 875

Answer 875

Harrison's groove, also known as Harrison's sulcus, is a horizontal groove along the lower border of the thorax corresponding to the costal insertion of the diaphragm; It is usually caused by chronic asthma or obstructive respiratory disease.

Question 876

DDx in asthma

Answer 876

Trransient early wheezing CF or bronchiectasis

Question 877

Dx of asthma

Answer 877

Skin-prick CXR rto rule out other conditions PEFR either diary or before and after inhaling bronchodilator Tests of lung funciton with spirometry gold standard can also trial asthma treatment to assess responsiveness

Question 878

Give 2 exmaples of SABAs

Answer 878

Salbutamol Terbutaline

Question 879

What is an anticholinergic bronchodilator

Answer 879

Ipratorpium bromide

Question 880

Give 3 examples of ICS

Answer 880

Budesonide Beclometasone Fluticasone Mometasone

Question 881

Give 2 examples of LABAs

Answer 881

Salmeterol Formoterol

Question 882

What drug class if theophylline

Answer 882

Methylxanthine

Question 883

Give an example of an oral steroid

Answer 883

Prednisolone

Question 884

What can be used for anti-IgE injection?

Answer 884

Omalizumab

Question 885

What age group is GORD common in?

Answer 885

INfancy due to inappropriate relaxation of the LOS as a result of functional immaturity

Question 886

Risk factors for GORD in infants?

Answer 886

Predominanly fluid diet Persistent horizontal posture Short intra-abdominal oesophageal length Severe reflex: CP other neurodevelopmental disorders, Prems with coextant BPD, following oesophageal atrsia/diaphragmatic hernia surgery

Question 887

Cx of GORD

Answer 887

FTT if sever vomiting Oesophagitis: haematemesis, discomfort on feeding, IDA Recurrent pulmonary aspiration: pneumonia, cough, wheeze Sandifer syndrome ATLEs

Question 888

Sandifer syndrome

Answer 888

The classical symptoms of the syndrome are spasmodic torticollis and dystonia.[3][4][5] Nodding and rotation of the head, neck extension, gurgling, writhing movements of the limbs, and severe hypotonia have also been noted.[3] Spasms may last for 1–3 minutes and may occur up to 10 times a day. Ingestion of food is often associated with occurrence of symptoms; this may result in reluctance to feed. Associated symptoms, such as epigastric discomfort, vomiting (which may involve blood) and abnormal eye movements have been reported. Clinical signs may also include anaemia.[2] Associated with GORD

Question 889

ATLE

Answer 889

An apparent life-threatening event (ALTE) describes an acute, unexpected change in an infant’s breathing, appearance, or behavior that is frightening to the parent or caretaker. It is not a specific diagnosis, but rather a “chief complaint” that brings an infant to medical attention.

Question 890

Ix in GORD

Answer 890

Clinical: no investigations required unless atypical history/cxs/ failure to respond 24h oesophageal pH monitoring Oesophageal endoscopy Contrast study to exclude other causes

Question 891

Mx of GORD

Answer 891

Majority resolve spontaneously by 12m Thicken feed 30o head up prone positioning after feeds Acid suppression (severe disease): Ranitidine (H2R antag) Omeprazole (PPI) Domperidone (enhances gastric emptying) Nissen's fundopplication

Question 892

Def: post nasal sinusitis

Answer 892

Infection of the paranasal sinuses Frontal sinusitis very rare in childhood as frontal sinuses have nt developed Caused by viral URTIs, occasionally secondary bacterial infection

Question 893

Mx post nasal sinusitis

Answer 893

Abx Analgesia Topical decongestants intranasal corticosteroids or antihistamines

Question 894

Transmision of TB in children?

Answer 894

Contract from adult, they are less likely to spread as disease is paucibacillary

Question 895

Signs/symptoms of TB

Answer 895

Priamry infection-\> dormancy-\> reactivation to post-priamry TB Systemic symptoms: FLAWS Cough

Question 896

Dx of TB

Answer 896

CXR: Ghon complex, hilar lymphadenopathy Sputum MCS (unobtainable from children \<8): Gastric washings on 3 consecutive mornings Mantoux Urinalysis LN CSF and radiological examination as appropriate IFNg release assays can help assess T cell response to TB antigens Coinfection with HIV makes dx difficult as Mnatoux and IGRA are both negative due to immunosuppression

Question 897

Ghon's complex

Answer 897

Ghon's complex is a lesion seen in the lung that is caused by tuberculosis. The lesions consist of a calcified focus of infection and an associated lymph node.

Question 898

Answer 898

Ghon complex

Question 899

Dx of TB gold standard

Answer 899

3 consecutive sputum samples stained with Ziehl-Neelsen for FAAB and cultured on Lowenstein-Jense Can take 4-8w Sensitivity can take a further 3-4w Empirical abx

Question 900

Mx of TB

Answer 900

RIPE Dexamethasone in tuberculous meningitis

Question 901

Mx of mantoux-positive but asymptomatic children

Answer 901

RI for 3m to prevent reactivation

Question 902

Prevention and contact tracing in TB

Answer 902

BCG Screen household

Question 903

Def: bronchiectassis

Answer 903

Permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall Caused by recurrent inflammation or infection of the airways. Occasionally begins in childhood following a severe lung infection or inhaling of a foreign object

Question 904

Risk factors for bronchiectasis

Answer 904

CF Host immunodeficiency Previous infections Congential diosrders of the bronchial airways Priamry ciliary dyskinesia

Question 905

Symptoms of bronchiectasis

Answer 905

Chronic cough with sputum Dyspnoea and fever Cyanosis, haemoptysis, fatigue Breath odor, weight loss, wheezing, clubbing

Question 906

Examination findings in bronchiectasis?

Answer 906

Crackles High-pitched inspiratory squeaks and ronchi

Question 907

Dx of bronchiectasis

Answer 907

CXR HRCT: gold standard FBC Sputum MCS Test if ?underlying disorder

Question 908

Mx of bronchiectasis

Answer 908

Exercise and improved nutrition Airway clearance therapy: PT and postural drainage Drugs Inhaled SABA Inaheld hyperosmolar agent: nebulised hypertonic saline Long term azithromycin

Question 909

Cx in inhaled foreing body?

Answer 909

Inflammation and infection Partial/total airway blockage Pneumonia Pneumothorax Subglottic oedema Lung abscesses Bronchiectasis

Question 910

Triad in inhaled foreign body

Answer 910

Coughing/choking Wheezing Unilateral reduced breath sounds (+respiratory distress)

Question 911

Causes of acute diarrhoea

Answer 911

Viral gastroenteritis Bacterial gastroenteritis (shigella, E Coli, Salmonella, campylobacter) Extraintestinal infections Antibiotic induced

Question 912

Normal stool pattern in a breat fed 0-4m old

Answer 912

2-4 per day (1-7=range) Yellow to golden, porrdigy consistency Infrequency is also normal (up to once per week)

Question 913

Normal stool pattern in bottle fed 0-4m old

Answer 913

2-3 per day Pale yellow to light brown pH7

Question 914

Normal stool pattern in 4m-1y/o

Answer 914

1-3 per day Darker yellow Firm

Question 915

Normal stool pattern \>1y/o

Answer 915

Formed like adult stool in odour and colour

Question 916

Ix in acute diarrhoe and indication

Answer 916

Stool microscopy and culture: blood/mucus (bacterial enteritis Stool immunoassay: hospitalised child (rotavirus) Blood count: high fever (?bacterial infection) Blood and urine culture, CXR: ?extraintestinal infeciton (bacterial)

Question 917

Age \<2y/o Watery stool Occasional pain Rare fits Vomiting common High fever common In winter

Answer 917

Rotavirus

Question 918

Age 1-5y Watery, blood, mucus, pus in stool Painful 10% fit Vomiting common High fever common Usually late summer

Answer 918

Shigella

Question 919

\<2 y/o Loose stool Painful Rare fits Vomiting common Rare fever Usually late summer

Answer 919

E Coli

Question 920

Any age Loose and slimy stool Painful Rare fits Vomiting common High fever common Usually late summer

Answer 920

Salmonella

Question 921

Any age Water, blood, mucus in stool Painful Rarely fits Rarely vomits Common fever Usually late summer

Answer 921

Campylobacter

Question 922

Common causes of chronic or recurrent diarrhoea Watery Fatty Bloody

Answer 922

Nonspecific diarrhoea Toddler diarrhoea Lactose intolerance Parasites: Giardia Cow's milk protein allergy Overflow diarrhoea in constipation CF Coeliac UC Crohn's

Question 923

Ix in chronic diarrhoea

Answer 923

Bloods: FBC ESR Coeliac Abs Stool: Occult blood Ova and parasites Reducing substances and pH Chymotrypsin Microscopy for fat globules Other: Urine MC+S Sweat test Breath hydrogen test Jejunal biopsy Barium meal and enema Endoscopy

Question 924

Answer 924

Question 925

Causes of vomiting in infants?

Answer 925

GORD Feeding problems Infection: Gastroenteritis RT/otitis media Pertussis UTI Meningitis Dietary protein intolerances Intestinal obstrucion: py sten Atresia Intussuception Malrotation Volvulus Duplication cysts Strangulated hernia Hirschprung Inborn errors of metabolism CAH Renal failure

Question 926

Causes of vomiting in preschool children?

Answer 926

Gastroenteritis Infeciton: RT UTI Meningitis Pertussis Appendicitis Intestinal obstruction: Intussuception Malrotation Volvulus Adhesions Foreign body: bezoar Raised ICP Coeliac Renal failure Inborn errors of metabolism Torsion of testis

Question 927

Causes of vomiting in school-age adolescents

Answer 927

Gastroenteritis Infections: pyelonephritis, septicaemia, meningitis ``` Peptic ulceration (H. pylori) Appendicits ``` Migraine Raised ICP Coeliac Renal failure DKA ETOH/drug ingestion Cyclical vomiting syndrome Bulimia/AN Pregnancy Torsion

Question 928

Cyclical vomiting syndrome

Answer 928

Cyclical vomiting syndrome (CVS) is a rare vomiting disorder most commonly seen in children, although it can affect adults too. Someone with CVS will frequently feel very sick and will vomit for hours, or even days, at a time. They will then recover from the episode and feel perfectly well, before experiencing another episode perhaps a month or so later. These vomiting attacks are not explained by an infection or other illness. CVS can affect a person for months, years or even decades. Symptoms can be so severe that some sufferers may need to stay in bed and be treated in hospital during an episode.

Question 929

What are the red flag symptoms in a vomiting child

Answer 929

Bile stained Haematemesis Projectile vomiting in first few weeks of life Vomiting at the end of paroxysmal coughing Abdominal tenderness, abdo pain Abdominal distension Hepatosplenogmegaly Blood in stool Severe dehydration or shock Bulging fontanelle or seizures FTT

Question 930

What are the red flag symptoms in a vomiting child Bile staind vomit

Answer 930

Intestinal obstruction

Question 931

What are the red flag symptoms in a vomiting child Haematemesis

Answer 931

Oesophagitis Peptic ulceration Oral/nasal bleeding

Question 932

What are the red flag symptoms in a vomiting child Vomiting at the end of paroxysmal coughing

Answer 932

Pertussis

Question 933

What are the red flag symptoms in a vomiting child Abdominal tenderness/pain on movement

Answer 933

Surgical abdomen

Question 934

What are the red flag symptoms in a vomiting child Abdominal distension

Answer 934

Intestinal obstruction including strangulated inguinal hernia

Question 935

What are the red flag symptoms in a vomiting child Hepatosplenomegaly

Answer 935

Chronic liver disease

Question 936

What are the red flag symptoms in a vomiting child Blood in stool

Answer 936

Intussuception Gastroenteritis: salmonella or campylobacter

Question 937

What are the red flag symptoms in a vomiting child Severe dehydration/shock

Answer 937

Severe gastroenteritis Systemic infection (UTI, septicaemia, meningitis) DKA

Question 938

What are the red flag symptoms in a vomiting child Bulging fontanelle or seziures

Answer 938

Raised ICP

Question 939

What are the red flag symptoms in a vomiting child FTT

Answer 939

GORD Coeliac Other chronic GI conditions

Question 940

Presentation of gastroenteritis

Answer 940

Sudden onset, \<7d Diarrhoea and vomiting \<3d Fever infectious contact/history of recent travel

Question 941

What is the length of adenoviral gastroenteritis?

Answer 941

\>14d

Question 942

What are the complications of gastroenteritis?

Answer 942

Dehydration (5-10%- dehydration, \>10%- shock) Changes in plasma Na

Question 943

What Na state leads to more recognisable signs of dehydration?

Answer 943

Hyponatraemia Increased H2O intake !cerebral oedema, fluid shift gives sign of dehydration

Question 944

What are the issues with hypernatraemic dehydration

Answer 944

Insensible water losses, signs of dehydration are less obvious There may be cerebral shrinkage Neurological symptoms may be prominenet e.g. hyperreflexia, hypertonia, jitters, convulsions, transient hyperglycaemia

Question 945

Mx of gastroenteritis

Answer 945

If no dehydration: Cotinue feeding, increase fluid intake, decrease fruit/juice/carbonated drinks Oral rehydration supplentations: low osmolarity e.g. diarolyte (50ml/kg often and in small amounts) If persists IV fluids 50ml/kg If in shock IV fluids Monitor electrolytes, urea, creatinine, glucose Abx if sepsis, immunocompromised or blood and mucus in stool Ampicillin, co-trimoxazole, cephalosporins Increase nutritional intake +/- Zn Advise re hand hygiene, not sharing towels, 48h isolation, no swimming for 2w and nutritional management

Question 946

What is post GE syndrome

Answer 946

Temporary lactose intolerance Can be invesatiageted with clinitest: non-absorbed sugar in stools Mx ORS then return Severe cases may require dietician referral

Question 947

IV fluids in shocked child

Answer 947

NG if possible NS 0.9% +/0 5% dextrose Bolus of 20ml/kg Replace fluid deficit (100ml/kg) + maintenance fluids

Question 948

How to calculate IV fluid replacement

Answer 948

1st 10kg: 100ml/kg/d 2nd 10kg: 50ml/kg/d Then 20ml/kg/d until 50kg Replace over \>48h, reduce Na \<0.5mM/l/hr

Question 949

What are the conditions that can mimic gastroenteritis

Answer 949

Systemic infeciotn: septicaemia, meningitis Local infection: RTI, otitis media, Hep A, UTI Surgical: Py sten, intussuception, acute appendicits, NEC, Hirschprung Metabolic: DKA Renal: HUS Other: coeliac, CMPI, adrenal insufficiency

Question 950

What are the clinical features of shock from dehydration in an infant

Answer 950

Decreased level of consciousness Sunken fontanelle Dry mucous membranes Eyes sunken and tearless Prolonged CRT Tachypnoea Tachycardia Weak peripheral pulse Pale or mottled skin Hypotension Sudden weight loss Reduce UO Cold extremities Reduced tissue turgor

Question 951

What are the red flags that help identify a child at risk of progression to shock?

Answer 951

Appears unwell or deteriorating Altered responsiveness Sunken eyes Tachycardia Tachypnoea Reduced skin turgor

Question 952

Answer 952

Question 953

Answer 953

Question 954

Answer 954

Question 955

Answer 955

Question 956

What is the intial fluid deficit in a child?

Answer 956

100ml/kg if shocked: 10% body weight 50ml/kg if not shocked: 5%

Question 957

What age group is appendicits less common in?

Answer 957

\<3y/o

Question 958

What is the timecourse of appendicits?

Answer 958

6-12h full thickness inflammation of the abdominal wall 24-36h gangrenous and perforation

Question 959

What are the complications of appendicitis

Answer 959

Perforation: omentum less well developed Appendiceal mass Abscesss

Question 960

Ix in appendicitis

Answer 960

Repeated obs USS (thickened, non-compressible appendix, increased blood flow)

Question 961

Mx of appendicitis

Answer 961

If no signs of perf, Abx and elective surgery If perforated- fluid resus, iv Abx prior to surger

Question 962

Complications of py sten

Answer 962

Hypochloraemic metabolic alkalosis, low Na, low K

Question 963

Ix in py sten

Answer 963

Test feed- gastric peristalsis L-\>R Feels like a knuckle in RUG If stomach is overdistended, should be emptied by NG tube to allow palpation USS

Question 964

Aetiology of py sten

Answer 964

Hypertrophy and hyperplasia of the pylorus muscle

Question 965

Hx in py sten

Answer 965

Projectile vomiting immediately or just after feed iNfant hungry immediately after vomit Constipation

Question 966

Symptoms of malrotation/volvulus

Answer 966

Obstruction +/- strangulation Bilious vomiting (1^st few days of life) Abdo pain Tenderness from peritonitis/ ischaemic bowel

Question 967

Aetiology of malrotation/volvulus

Answer 967

Mesentry not flexed at the DJ flexure or IC region, shorter base means they are predisposed to volvulus Ladd bands (peritoneal bands) obstructing the duodenum or volvulus

Question 968

Cx of volvulus

Answer 968

Strangulation

Question 969

Ix in malrotation

Answer 969

If dark green vomiting, contrast study to assess rotation

Question 970

Mx of malrotation/volvulus

Answer 970

Urgent laparotomy if vascular compromise Untwist volvulus Mobilise duodenum Malrotation not corrected but mesentry broadened

Question 971

Intestinal malrotation

Answer 971

Intestinal malrotation is a congenital anomaly of rotation of the midgut (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result: the small bowel is found predominantly on the right side of the abdomen the cecum is displaced (from its usual position in the right lower quadrant) into the epigastrium - right hypochondrium the ligament of Treitz is displaced inferiorly and rightward fibrous bands (of Ladd) course over the vertical portion of the duodenum (DII), causing intestinal obstruction. the small intestine has an unusually narrow base, and therefore the midgut is prone to volvulus (a twisting that can obstruct the mesenteric blood vessels and cause intestinal ischemia).

Question 972

How can the causes of acute abdominal pain be classified?

Answer 972

Intra-abdominal- Surgical Medical Extra-abdominal

Question 973

What are the surgical causes of acute abdo pain

Answer 973

Acute appendicitis Intestinal obstruction Inguinal hernia Peritonitis Inflamed Meckel diverticulum Pancreatitis Trauma

Question 974

What are the medical causes of acute abdo pain

Answer 974

Non-specific abdo pain GE UT: UTI, pyelonephritis, hydronephrosis, renal calculus HSP DKA SCD Hepatitis IBD Constipation Recurrent abdo pain of childhood Gynaecological Psychological Lead poisoning Acute porphyria Idiopathic

Question 975

What are the extra-abdominal causes of acute abdo pain?

Answer 975

URTI Lower lobe pneumonia Torsion of the testis Bony

Question 976

Def: mesenteric adenitis

Answer 976

Isolated, non-specific inflammation of the mesenteric LNs Dx of exclusion made when LNs are seen on laparotomy or laproscopy when the appendix is normal

Question 977

Symptoms of mesenteric adenitis

Answer 977

Non-specific, self-limiting abdo pain D+V Nausea Fever

Question 978

Ix in mesenteric adenitis

Answer 978

USS FBC CRP WCC To exclude appendicitis Gold standard: laparoscopic visualisation of large LNs

Question 979

Aetiology of mesenteric adenitis

Answer 979

Likely to be viral infection Appendicitis UTI

Question 980

Mx of mesenteric adenitis

Answer 980

Conservative Painkillers

Question 981

Considerations re bacterial spp causing UTI

Answer 981

E COli Proteus: phosphate stones Pseudomonas: ?structural abnormality Strep faecalis

Question 982

What are the risk factors for developing UTIs

Answer 982

Infrequent voiding Vulvitis Incomplete micturition-\> residual post-mic bladder volume Constipation Neuropathic bladder VUR Posterior urethral valves

Question 983

TIN CAN MED DIPS

Answer 983

Trauma Infection Inflammation Neoplastic Circulatory Congeital Autoimmune Allergy Nutrition Metabolic Musculoskeletal Endocrine Drugs Degenerative Iatrogenic Psychosomatic Structural

Question 984

Symptoms of UTI in infants

Answer 984

Fever, vomiting, lethargy, off feeds, FTT, irritable, jaundice, septicaemia, offensive urine, febrile convulsions

Question 985

Neonate=

Answer 985

Baby from birth to 4w

Question 986

Infant=

Answer 986

4w-1y

Question 987

Toddler=

Answer 987

1-2y

Question 988

Pre-school=

Answer 988

2-5y

Question 989

School-age=

Answer 989

Older child

Question 990

Teenager=

Answer 990

Adolescent

Question 991

Symptoms of UTI in children

Answer 991

Dysuria, frequency, abdo pain/loin tenderness, fever +/- rigors, lethargy, anorexia D+V, Blood/offesnive/cloudy/recurrent enuresis Febrile convulsion Dysuria without systemic symptoms= lower UTI

Question 992

Ix in typical UTI

Answer 992

Urine dipstick: WCC nitrites MC+S

Question 993

What is an atypical UTI?

Answer 993

If it leads to sepsis Poor flow Abdominal mass Raised creatinine non-E. Coli No Abx response

Question 994

Ix in atypical/recurrent UTI

Answer 994

USS: structural abnormalities, renal defects, scarring, check for posterior urethral valve in boys. If something seen MCUG (\<3y) or MAG3 for obstruction/VUR DMSA for scars 3/12 post UTI

Question 995

What is the difference between a DMSA, MAG3 and MCUG?

Answer 995

DMSA is an injection of radioactive dye, then they scan the kidneys and the machine picks up the dye. From this they can see how much dye each kidney is holding on to (it holds in the healthy areas of the kidneys) so they can see a precentage of each kidneys function and see which areas may be scarred from infections. A Mag3 is similar in the way that they inject a dye, its just a coloured fluid this time then they scan the kidneys and bladder to watch how the fluid drians through the kidneys. They can see if any fluid is refluxing back up, if there are any blockages and they can see if the bladder is emptying itself properly. An Mcug is agin similar thought here is no injection. The dye is inserted through a catheter, they scan and watch how the bladder drains.

Question 996

What are the possible methods for sampling urine?

Answer 996

Clean catch Adhesive plastic bag on perineum Urethral catheter Suprapubic aspiration

Question 997

Antibiotic prophylaxis in UTI

Answer 997

Should not be routinely recommended in infants and children following first time UTI CAn be considered with in children with recurrent UTI Asyomptomatic bacturia should not be treated Can use low dose trimethoprim or nitrofurantoin

Question 998

Advice to parents re UTI

Answer 998

High fluid intake High urine output Regular voiding to complete micturition Address constipation Good perineal hygiene Probiotics

Question 999

Def: recurrent abdominal pain

Answer 999

Pain sufficeint to interrupt normal activities that lasts for \>3m

Question 1000

Symptoms of recurrent abdominal pain

Answer 1000

Chronic periumbilical pain in an otherwise well child

Question 1001

Ix in recurrent abdominal pain

Answer 1001

Exclude organic issues As well as Social/psychiatric/psychological causes= insepction of perineum, check growth Abdo USS stones at PUJ

Question 1002

Mx of recurrent abdominal pain

Answer 1002

Family/school input ?abuse/stress School avoidance Address family's understanding and concerns Need to explain to child what the condition is about Explain prognosis

Question 1003

Px of recurrent abdominal pain

Answer 1003

50% symptoms resolve rapidly 1/4 of symptoms take months 1/4 return in adulthood as migraine, IBS

Question 1004

What are the symptoms and signs that suggest organic disease in a child with recurrent abdo pain

Answer 1004

Epigastric pain at night, haematemesis (duodenal ulcer) Diarrhoea, weight loss, FTT, blood in stools (IBD) Vomiting (pancreatitis) Jaundice (liver disease) Dysuria, secondary enuresis (UTI) Bilious vomiting and abdo distension (malrotation)

Question 1005

Def: intussuception

Answer 1005

Invagination of proximal bowel into a distal segment. Most commonly ileum passing into caecum thru ileocaecal valve. Most common cause of obstruction after neonatal period, peak age between 3 months and 2 years

Question 1006

Cx of intussuception

Answer 1006

stretching and constriction of the mesentery à venous obstruction à engorgement and bleeding of the bowel mucosa, fluid loss à perforation, peritonitis, necrosis

Question 1007

Aetiology of intussuception

Answer 1007

Viral infexn à enlargement of Peyer’s patch may form a lead point. IN kids \>2 à emckel diverticulum or polyp

Question 1008

What is the most common cause of obstruction peaking between 3m and 2y?

Answer 1008

Intussuception

Question 1009

Symptoms in intussuception

Answer 1009

* Paroxysmal, sever colicky pain an pallor esp around the mouth * Draws up leg, initialy recoveres between painful episodes butsubsequently increasingly lethargic * Refuses feeds, vomits can be bile=stained * Sausage-chaped mass * Redcurrant jelly stool + blood-stained mucus (late sign) Abdo distension + shock

Question 1010

Ix in intussuception

Answer 1010

USS

Question 1011

Mx of intussuception

Answer 1011

IV fluid resuscitaiton: pooling of fluid in gut can precipitate hypovolaemic shock Reduction by air insufflation unless signs of peritonitis In which case, sx

Question 1012

Answer 1012

Question 1013

Draw the constipation management algorithim

Answer 1013

Involve dieticians Offer advice on behavioural interventions for children started on maintenance laxative drug treatment. The intervention should be consistent with the child's age and stage of development and may include: Scheduled toileting — encourage the child to try and open their bowels at pre-planned intervals or activities, such as after each meal for five minutes, or before bedtime. Use of a bowel habit diary — to track the frequency and consistency of stool. The ERIC Toilet Tool Wallchart may be helpful. Use of encouragement and rewards systems — such as star charts incorporated into toileting routines, to help praise good behaviour such as visiting the toilet. Give diet and lifestyle advice and information on recommended fluid intake if needed, in combination with advice on the early use of laxatives and behavioural interventions. Foods with a high fibre content include fruit, vegetables, high-fibre bread, baked beans, and wholegrain breakfast cereals. Do not recommend unprocessed bran (which may cause bloating and flatulence and reduces the absorption of micronutrients) or fibre supplements.

Question 1014

Answer 1014

Question 1015

Def: fissure in ano

Answer 1015

Tearing of tissue around the anal sphincter

Question 1016

Risk factors for fissure in ano

Answer 1016

Trauma from hard stools, diarrhoea, snal instrumentation, low fibdre intake

Question 1017

Symptoms of fissure in ano

Answer 1017

Severe anal pain – tearing/cutting/burning during or after defecation; PR bleed of bright red blood, itchy bum

Question 1018

Symptoms of crohn's in children

Answer 1018

Calssically: abdo pain, diarrhoea, weight loss, ?bloody stools Growth failure, delayed puberty Generally ill: fever, lethargy, weight loss Extra-intestinal: oral lesions, perianal skin tags, anterior uveitisi, arthralgia, erythema nodosum, pyoderma gangrenosum

Question 1019

Endoscopic and histological findings on biopsy Endoscope: actuely inflamed, thickened bowel à strictures of bowel and fistulae between adjacte bowel loops or with other organs Histology: non-caseating epithelioid cell granulomata Small bowel imaging: narrowing, fissureing, mucosal irregularitites and thickened bowel wall

Answer 1019

Crohn's

Question 1020

Mx of Crohn's

Answer 1020

Nutritional therapy: normal diet replaced with whole protein modular feeds (polymeric diet) for 6-8/52 if unresponsive, give systemic steroids Remission maintenance: azathioprine, 5-mercaptopurine, methotrexate Overnight NG feed can be used to correct growth failure Requires complex MDT Mx

Question 1021

Cx of Crohn's

Answer 1021

Obstruction Fistulae Abscesss Severe localised disease-\> Sx Post Sx: risk of short bowel syndrome: malodorous diarrhoea, vitamin/mineral malabsorption

Question 1022

**Endoscopy**: Confluent colitits extending from rectum proximally, for a vriable elgnth cf adults where diseae mainly confied to distal colon (kids have pancolitits) **Histology**: mucosal inflammation, crypt damage, ulceration Small bowel imaging: rule out the extra-colonic inflammation that suggests Crohns instead

Answer 1022

UC

Question 1023

Mx of UC

Answer 1023

Aminosalicylates (balsalazide, mesalazine for induction and maintenance) Topical steroids can be used if it is refined to the rectum/sigmoid Aggressive/extesnive disease requires systemic steroids and immunomodulatory drugs for maintenance Severe fulminating disease: fluid resusc and steroids, ciclosporin i funresponsive

Question 1024

Def: meckel diverticulum

Answer 1024

Ileal remnant of the vitello-intestina duct Contains ectopic gastric mucosa or pancreatic tissue

Question 1025

Symptoms of a meckel diverticulum

Answer 1025

Asymptomatic or present with severe rectal bleeding- niehter bright red nor true malaena. Can also present as intussusception, volvulus or diverticulitis

Question 1026

Ix in meckel diverticulum

Answer 1026

Technecium scan – increased uptake by gastric mucosa

Question 1027

Mx MEckel diverticulum

Answer 1027

Sx

Question 1028

Draw the mx of UTI

Answer 1028

Question 1029

Draw scanning following UTI \<6m 6m-3y \>3y

Question 1030

Def: tension headache

Answer 1030

Symmetrical diffuse headache with gradual onset with mild to mederate severity described as the feeling of a tight band across forehead. Bilateral Associated with stress

Question 1031

Red flags in headache

Answer 1031

Acute, severe pain Fever Worse when lying down, coughing, straining Waking child Altered mental state Vomiting/nausea Focal neurology Abnormal cerebellar signs Bradycardia Poor school performance/developmental regression Consistent unilateral pain Cranial bruit HTN Papilloedema FTT

Question 1032

How to auscultate for cranial bruit

Answer 1032

A bruit should be listened for, in quiet surroundings, over the skull and eyeballs, the latter situation being the most favourable for hearing the softest ones. The patient should be asked to close both eyes gently and the stethoscope firmly applied over one eye. During auscultation the other eye should be opened as in this way there is considerable diminution of eyelid flutter, which may cause confusion if rhythmical. Auscultation is then carried out over the other eye in a similar manner. If a murmur is not readily heard the patient should be asked to hold his breath. Finally auscultation should be carried out over the temporal fossæ and mastoid processes.

Question 1033

Def: migraine

Answer 1033

Unilateral or bilateral pulsating headache which may or may not be characterised by an aura

Question 1034

Features of migraine without aura

Answer 1034

Usually bilateral pulsating headache, Accompanied by GI disturbances and photophobia or phonophobia. Lasts 1-72hrs.

Question 1035

Features of migraine with aura

Answer 1035

Negative phenomena, such as hemianopia (loss ofhalf the visual field) or scotoma (small areas of visual loss) OR Positive phenomena such as fortification spectra (seeing zigzag lines).

Question 1036

DDx migraine

Answer 1036

Primary: migraine, cluster headache Secondary headache: head/neck trauma, raised ICP, idiopathic intracranial HTN, SOL, vascular malformation, infection, sinusitis, psychiatric

Question 1037

IHS criteria for migraine in children (

Answer 1037

Question 1038

Mx of migraine

Answer 1038

Conservative often more effective: identification of triggers and predisposing factors. Behavioural management: routine around sleeping and eating Acute attack: Paracetamol/ ibuprofen Anti-emetics: domperidone, prochlorpromazine. 5HT agonistst can be used if simple analgesia fails e.g. sumitriptan. (Specialist only) Prophylaxis (if severely impacting on school life): Pizotifen (5HT antagonist) can cause weight gain and sleepiness Beta blockers: propranolol (CI-ed in asthma) Na channel blockers: Topimarate

Question 1039

Sympto9ms of myopia/hypermetropia

Answer 1039

Headache when trying to read or reading from afar.

Question 1040

Ix in myopia/hypermetropia

Answer 1040

Vision test, optician assessment Exclude other organic pathology

Question 1041

Features of post-ictal headache

Answer 1041

Post-seizure headache with features of tension type or migraine headache developing within 3 hours of a partial/generalised seizure resolving within 72h of seizure

Question 1042

Def: SOL

Answer 1042

Solid tumour, hydrocephalus, haemorrhage

Question 1043

What are the causes of non-communicating hydrocephalus?

Answer 1043

Obstruction of ventricular system e.g. Chiari malformation Aqueduct stenosis Atresia of outflow foramina of fourth ventricle Posterior fossa neoplasm AVM Intraventricular haemorrhage

Question 1044

Causes of communicating hydrocephalus

Answer 1044

I.e. failure to reabsorb CSF: SAH Meningitis

Question 1045

Early effects of SOL

Answer 1045

Raised ICP Neurological problems Mets Death

Question 1046

Late effects of SOL

Answer 1046

Neurological disability Growth problems Endocrine Neuropsychological Educational

Question 1047

What are the features of ICP?

Answer 1047

* headache worse when lying down, coughing or straining * headache waking child * confusion * vomiting/nausea * cranial nerve abnormalities: visual defects/squint, diplopia, facial palsy etc * abnormal coordination * abnormal gait * bradycardia * poor school performance/regression of developmental skills * consistent, unilateral pain * cranial bruit -\> arteriovenous malformation * hypertension * torticollis – neck twisting * papilloedema (late feature) * failure to thrive

Question 1048

Features of hydrocephalus in younger children?

Answer 1048

* accelerated head growth * bulging fontanelle * separated sutures * dilated scalp veins * “setting sun” eyes – downward deviation of eyes * “cracked pot” sound on skull percussion * skull transillumination in older children, hydrocephalus presents as raised ICP

Question 1049

Imaging in SOL

Answer 1049

If red flags present: MRI For hydrocephalus: cranial USS, CR or MRI: monitor head circumference MRI for all children complaining of persistent back pain

Question 1050

for all children complaining of persistent back pain?

Answer 1050

MRI

Question 1051

Features of tension headache

Answer 1051

Band like, constricting Towards end of day No associated features Normal physical examination

Question 1052

Features of migraine

Answer 1052

Throbbing, unilateral headache No specific timing N+V, photophobia, FHx Normal

Question 1053

Characteristics of raised ICP

Answer 1053

Worse on lying down, may be localised to site Early morning or waking at night Vomiting without nausea Slow pulse, high BP, papillodema, enlarging head circumference, focal signs

Question 1054

What are the types of fits seen in infancy

Answer 1054

Apnoea and ALTEs Febrile convulsions Breath-holding Infantile spasms Epilepsy Hypoglycaemia and metabolic conditions

Question 1055

Types of fits seen beyond infancy

Answer 1055

Febrile convulsions Breath-holding: cyanotic spells, pallid spells Night terrors Epilepsy BPV

Question 1056

Fits seen in school age

Answer 1056

Epilepsy Syncope Hyperventilation Hysteria Tics

Question 1057

Characteristics of ALTE

Answer 1057

Usually found limp or twitching No apparent precipitating event EEG may be helpful

Question 1058

Feature of breath-holding spells (cyanotic)

Answer 1058

Stops breathing, becomes cyanotic and extends, may lose consciousness. Becomes limp and breathes normally, no postictal state Always precipitated by crying from pain or anger EEG not indicated

Question 1059

Features of reflex anoxic spells (pallid)

Answer 1059

Turns pale and collapses, rapid recovery Precipitated by head or other minor injury EEG not indicated for dx

Question 1060

Characteristic features of night terrors

Answer 1060

Wakes from sleep disorientated and frightened. May have autonomic signs Precipitating event: sleep EEG not required for Dx

Question 1061

Characteristic features of BPV

Answer 1061

Sudden unsteadiness Frightened and clings to parent No postictal state EEG not required

Question 1062

Features of infantile spasms

Answer 1062

Jack-knife spasms occuring in clusters with developmental regression Often occur on waking Hypsarrhythmia on EEG

Question 1063

Criteria for febrile convulsions

Answer 1063

T \>38 \<6y/o no CNS infeciton/inflammation No acute systemic metabolic abnormality No history of previous afevrile seizures

Question 1064

What are the complications of febrile convulsions

Answer 1064

Complex febrile seizures: 4-12% risk of subsequent epilepsy

Question 1065

Symptoms of febrile convulsions

Answer 1065

\<20 mins generalised tonic clonic seizure

Question 1066

How can the causes of convulsions be classified?

Answer 1066

Epileptic Non-epileptic

Question 1067

What are the causes of epileptic seizures

Answer 1067

Idiopathic Tumour Cerebral dysgenesis Vascular occlusion Cerebral damage (congenital infection, hypoxic-ischaemic encephalopathy, IVH)

Question 1068

What causes 70-80% of epilepsy?

Answer 1068

Idiopathic

Question 1069

What are the non-epileptic causes of seizure

Answer 1069

trauma, metabolic (hypoglyc, hypoCa, hypoMg, hypoNa, hyperNa), meningitis, encephalitis, poisons

Question 1070

Ix in febrile convulsions

Answer 1070

BM ?Cause of fever, septic screen Bloods Exclude meningitis

Question 1071

What proportion of children will have recurrance of febrile seizure?

Answer 1071

30%

Question 1072

Advice for parents with febrile convulsion in child?

Answer 1072

What febrile seizures are. How to treat fever at home - remove excess clothing, give fluids, give antipyretics if the child is uncomfortable. Tepid sponging or excessive cooling are not recommended. Check for a non-blanching rash, check for dehydration and stay with the child at night. First aid if the child has a fit - position; do not put anything in their mouth. When to call 999/112/911 ambulance - a seizure lasting more than five minutes. When and how to seek urgent medical advice - any seizure, serious symptoms such as non-blanching rash, lack of normal alertness, dehydration, the child getting worse, the parent worried and fever for more than five days.

Question 1073

Mx of febrile convulsions

Answer 1073

Supportive Treat underlying cause of pyrexia Abx if LP contraindicated NB: antipyretics don't prevent febrile seizures

Question 1074

What do for child with febrile convulsions if there is a history of prolonged seizures/epilepsy/poor access to hospital

Answer 1074

Consider giving parents rectal diazepam or buccal midazolam

Question 1075

Confirmatory investigations in breath-holding spells?

Answer 1075

Normal EEG

Question 1076

What is the difference between a generalised and a focal seizure

Answer 1076

Generalised involves both hemispheres Focal one hemisphere

Question 1077

Def: status epilepticus?

Answer 1077

Status epilepticus = \>30 mins, or repeated seizures without recovery of consciousness for 30mins

Question 1078

What are the features of focal seizures?

Answer 1078

Usually asymmetric, may or may not have LOS, may proceed to tonic-clonic Frontal: motor cortex, clonic movements Temporal: commonest, aura, automatisms, deja-vu +/- impaired consciousness Occipital- vision Parietal- dysaesthesias

Question 1079

Features of generalised seizures

Answer 1079

Always LOC, no warning, symettrical Absecnce: transient LOC with flickering eyelids Myoclonic: brief, repetitive jerking movements of limbs, neck, trunk Tonic- generalised increased tone Tonic-clonic: rigid tonic phase (with cyanosis) then clonic (rhythmical muscle contraction) and tongue biting, incontinence, followed by fatigue Atonic

Question 1080

Ix in epilepsy

Answer 1080

Examination: check for neurocutaenous syndrome EEG: NB may have normal EEG or may show neuronal hyperexcitability or asymmetry If normal consider sleep deprived record or 24h ambulatory MRI and CT are not routine unless focal signs +/- PET Metabolic investigaitons if there is associated developmental regression

Question 1081

Mx of epilepsy

Answer 1081

Explanation and advice AED treatment Stop AEDs after 2 yeasr seziure free Can drive if \>1y seizure free

Question 1082

Atonic seizure

Answer 1082

Often combined with a myoclonic jerk followed by transient loss of muscle tone causing a sudden fall to the floor or droop of the head

Question 1083

Treatment of West syndrome?

Answer 1083

Vigabatrin or corticosteroids Many subsequently develop LD or epilepsy

Question 1084

Features of Lennox-Gastaut Syndrome

Answer 1084

Seen in 1-3y/o Multiple seizure types, mostly drop attacks (astatic seizures), tonic seizures and atypical absences Neurodevelopmental arrest or regression and behaviour disorder Often other complex neurological problems Px is poor

Question 1085

Features of childhood absence epilepsy

Answer 1085

Stare momentarily and stop moving, may twitch eyelids or hands. Lasts \<30s. Child has no recall Normal developmentally 2/3rds female Can be induced by hyperventilation: ask children to blow on a piece of paper for 2-3 mins. Good Px

Question 1086

Features of BECTS

Answer 1086

Benign Epilepsy with centritemproal spikes 4-10y Tonic-clonic seizures in sleep or simple focal seziures with awareness of abnormal feelings. 15% of all childhood epilepsies EEG shows focal sharp waves from the Rolandic or centrotemproal area

Question 1087

Features of early onset benign childhood occipital epilepsy

Answer 1087

1-14 y.o Younger children: periods of unresponsiveness, eye deviation, vomiting and autonomic features Older children: headache and visual disturbances including distortion of images and hallucinations UNcommon EEG shows occcipital discharges Remits

Question 1088

Features of juvenile myoclonic epilepsy

Answer 1088

Adolescence-adulthood Myoclonic seizures but generaelised tonic-clonic and absences may occur Characteristic EEG Good response to treatment

Question 1089

First line Rx in tonic-clonic seizures

Answer 1089

VPA, carbamazepine

Question 1090

Second line Rx in tonic-clonic

Answer 1090

Lamotrigine, topiramate

Question 1091

First line Rx in absence seziures

Answer 1091

Valproate Ethosuximide

Question 1092

Second line Rx in absence

Answer 1092

Lamotrigine

Question 1093

First line Rx in myoclonic

Answer 1093

Valproate

Question 1094

Second line Rx in myoclonic

Answer 1094

Lamotrigine

Question 1095

First line Rx in focal seizures

Answer 1095

Lamotrigine most effective Carbamazepine Valproate

Question 1096

Second line Rx in focal seizures

Answer 1096

Topiramate Levetiracetam Oxcarbazepine, gabapentin, tiagabine, vigabatrin

Question 1097

ADEs in valpriate

Answer 1097

Weight gain Hair loss Rarely idiosyncratic liver failure

Question 1098

ADEs in carbamazepine

Answer 1098

Rash Neutropenia Hyponetraemia Ataxia Liver enzyme induction

Question 1099

ADEs in vigabatrin

Answer 1099

Restriction of visual fields which has limited its use Sedation

Question 1100

ADEs in lamotrigine

Answer 1100

Rash

Question 1101

ADEs in ethosuximide

Answer 1101

N+V

Question 1102

ADEs in topiramate

Answer 1102

Drowsiness, withdrawal and weight loss

Question 1103

ADEs in gabapentin

Answer 1103

Insomnia

Question 1104

ADEs in leveritacetam

Answer 1104

Sedation

Question 1105

Parital seizures Ix

Answer 1105

Imaging studies

Question 1106

Mx of status epilepticus

Answer 1106

Question 1107

Def: status epilepticus

Answer 1107

Seizures \>5 mins Or with no regaining of consciousn ess between them

Question 1108

NB for Rx used in status epilepticus

Answer 1108

All of the drugs may cause or compound pre-existing respiratory depression and thus mechanical ventilation may subsequently be required

Question 1109

Features of reflex anoxic seizure

Answer 1109

* infants/toddlers * pain/discomfort esp minor head trauma, cold food, fright/fever * pale, lose consciousness, may induce generalised tonic-clonic * due to cardiac asystole from vagal inhibition * spontaneous resolution

Question 1110

How to use rectal diazepam

Answer 1110

Knee-chest position on side INsert nozzle gently through anus up to hilt of spout Squeeze contents of tube into child's rectum over 2-3 minutes Remove applicator and lie child in recovery position

Question 1111

How to use buccal midazolam

Answer 1111

Liquid into the side of the mouth between the gums and cheeks Given slowly using a plastic syringe Divide dose- one on one side, one on the other Watch for reduction of breathing or cessation of seizure activity

Question 1112

Corticospinal tract disorders causing seizures

Answer 1112

Cerebral dysgenesis Global hypoxia-ischaemia Arterial ischaemic stroke Cerebral tumour Acute disseminated encephalomyelitis Post-ictal paresis Hemiplegic migraine

Question 1113

Basal ganglia disorders causing seizures

Answer 1113

Acquired brain injury: Acute and profound hypoxia-iscahemia, CO poisoning, post cardiopulmoanry bypass chorea Post-streptococcal chorea Mitochondrial cytopathis Wilson's HD

Question 1114

Cerebellar disorders causing seizures

Answer 1114

Acute: medication and drugs Post-viral: esp varicella Posterior fossa lesions or tumours e.g. medulloblastoma Genetic and degenerative disorders e.g. friedrich ataxia, ataxic cerebal palsy

Question 1115

Causes of an acute painful limp in 1-3 y/o

Answer 1115

Infection: septic arthritis, osteomyelitis of the hip or spine Transient synovitis Trauma: accidental or NAI Malignant disease: leukaemia, neuroblastome

Question 1116

Causes of a chronic and intermittent limp in a 1-3y/o

Answer 1116

DDH Talipes Neuromuscular e.g. CP JIA

Question 1117

Causes of an acute painful limp in 3-10y/o

Answer 1117

Transient synovitis Septic arthritis/osteomyelitis Trauma and overuse injuries Perthes disease (acute) JIA Malignant disease e.g. leukaemia Complex regional pain syndrome

Question 1118

Causes of chronic and intermittent limp in 3-10y/o

Answer 1118

Perthes disease (chronic) Neuromuscular disorders e.g. DMD JIA Tarsal coalition

Question 1119

Talipes

Answer 1119

Club foot or clubfoot, also called congenital talipes equinovarus (CTEV), is a congenital deformity involving one foot or both. The affected foot appears to have been rotated internally at the ankle.

Question 1120

Tarsal coalition

Answer 1120

A tarsal coalition is an abnormal connection that develops between two bones in the back of the foot (the tarsal bones). This abnormal connection, which can be composed of bone, cartilage, or fibrous tissue, may lead to limited motion and pain in one or both feet.

Question 1121

Causes of an acute painful limp in 11-16y/o

Answer 1121

Mechanical trauma Slipped femoral epihpysis Avascular necrosis of the femoral head Reactive arthritis JIA Septic arthritis/osteomyelitis Osteochondritis dissecans of the knee Bone tumours and malignancy Complex regional pain syndrome

Question 1122

Causes of a chronic and intermittent limp in 11-16y/o

Answer 1122

Slipped femoral epihpysis (chronic) JIA Tarsal coalition

Question 1123

Answer 1123

Question 1124

Answer 1124

Question 1125

Causes of swollen joints Trauma Infection Reactive arthritis Vasculitis Collagen vascular disease Haematological GI Malignancy

Answer 1125

Trauma Septic arthritis, viral Post-streptococcal or gastrointestinal infections HSP JIA, SLE Leukaemia, haemophilia, SCD UC, Crohn's Leukaemia

Question 1126

Ix in leg pain

Answer 1126

FBC: leukaemia, infections, colalgen vascular disease Plasma viscosity: infections ESR: collagen vascular disease CRP XR: tumours, infection, trauma, avascular necrosis, leukaemia, slipped capital femoral epiphysis Bone scan: osteomyelitis, stress factures, malignant tumours Muscle enzymes: damage to muscle cells

Question 1127

Ix in swollen joint

Answer 1127

FBC: infection, collagen vascular disease ESR and plasma viscosity Blood culture: septic arthritis ASO titre: indicative of recent streptococcal infection Viral titres RF and ANA: negative in most forms of JIA XR Joint aspiration: MC+S

Question 1128

Causesof polyarthritis Infection IBD Vasculitis Haematological Malignant CTD Other

Answer 1128

Bacterial: septcaemia, septic arthritis, TB Viral: rubella, mumps, adenovirus, coxsackie B, herpes, hepatitis, parvovirus Other: mycoplasma, lyme disease, ricektsia Reactive: post-strep, post-GI Rheumatic fever IBD: UC, crohn's Vasculitis: HSP, kawasaki Malignant: leukaemia, neuroblastoma CTD: JIA, SLE, dermatomyositis, MCTD, PAN CF

Question 1129

What are the types of JIA?

Answer 1129

Systemic Polyarticular Pauciarticular

Question 1130

Features of systemic JIA

Answer 1130

Large and small joints M\>F ANA negative No iridocyclitis 25% have severe arthririts (Most commonly causes severe arthritis)

Question 1131

Features of polyarticular JIA

Answer 1131

Large and small joints affected F\>M RhF negative, ANA may be positive No iridocyclitis 12% severe arthritis

Question 1132

Iridocyclitis and joint pain?

Answer 1132

Probably pauciarticular JIA

Question 1133

Features of pauciarticular JIA

Answer 1133

\<5 joints, usualyl large F\>M Rhf negative, ANA positive At high risk of iridocyclitis No severe arthritis usually

Question 1134

Def: reactive arthritis

Answer 1134

Most common form of arthritis in childhood A seronegative spondyloarthropathy that occurs following **gastrointestinal and genitourinary infection**

Question 1135

Reiter's syndrome triad

Answer 1135

See, pee, climb a tree Non-infectious urethritis Arthritis Conjunctivitis

Question 1136

Causes of reactive arthritis in children Adolescents

Answer 1136

_Children_: **enteric bacteria** (Salmonella, Shigella, Campylobacter, Yersinia) _Adolescents_: **STIs** (chlamydia, gonococcus) Mycoplasma, Lyme disease

Question 1137

Signs/symptoms of reactive arthritis

Answer 1137

1-4w post intiial infection Transient (\<6w) joint swelling, normally ankles or knees Low grade feber, malaise, fatiuge Urethritis: frequency, dysuria, urgency Conjunctivitis: erythema, burning, tearing, photophobia

Question 1138

Examination in reactive arthritis?

Answer 1138

Pain swelling, heat, redness and restricted movement in the joints: Asymmetric oligoarthritis affecting the **weight-bearing** joints

Question 1139

Mx of Reiter's

Answer 1139

No curative treatment Symptomatic NSAIDs: indomethacin Corticosteroids Abx if chlamydia related DMARDs only given if NSAIDs ineffective or contraindicated

Question 1140

Ix in Reiters

Answer 1140

(CLINICAL DIAGNOSIS) X-ray (normal) FBC, ESR, CRP (acute-phase reactants are normal/mildly elevated) ANA, Rheumatoid factor Urogenital and stool cultures Anthrocentesis with synovial fluid analysis

Question 1141

Def: osteomyelitis

Answer 1141

Infection of the **metaphysis** of long bones Most commonly **distal femur** and **proximal tibia** URGENT DIAGNOSIS AND TREATMENT REQUIRED

Question 1142

Aetiology of osteomyelitis

Answer 1142

Haematogenous spread or direct spread from an infected wound

Question 1143

Most common cause of osteomyelitis?

Answer 1143

Staph aureus Strep and HiB (if not immunised)

Question 1144

Most common cause of osteomyelitis? in sickle cell

Answer 1144

Staph and salmonella

Question 1145

Cxs of osteomyelitis

Answer 1145

May spread to cause **septic arthritis** (if capsular attachment is below metaphysis, as in hip) Bone necrosis, chronic infection with a discharging sinus, limb deformity and amyloidosis

Question 1146

Signs/symptoms of osteomyelitis

Answer 1146

Acute febrile illness Markedly painful, immobile limb (pseudoparesis)-\> moving limb causes severe pain Swelling and tenderness directly over infected site Erythematous and woarm May be a sterile effusion of an adjeacent joint Insidious onset in infants

Question 1147

Ix in osteomyelitis

Answer 1147

**Blood cultures** – usually positive **Acute phase reactants** (WBC,CRP, ESR) – significantly elevated **X-ray** – INITIALLY: normal 7-10 DAYS: subperiosteal new bone formation, bone rarefaction (periosteal elevation and radiolucent necrosis) **Ultrasound** – periosteal elevation at presentation **MRI** – subperiosteal pus and purulent debris in bone. Helps differentiate from soft tissue infection **Radionucleotide bone scan** – identify site of infection

Question 1148

XR in osteomyelitis

Answer 1148

Initially normal 7-10d: subperiosteal new bone formation, bone rarefaction (periosteal elevation and radiolucent necrosis)

Question 1149

Answer 1149

Osteomyelitis

Question 1150

Mx of osteomyelitis

Answer 1150

Abx for several weeks: IV until normal acute phase reactants, then oral Aspiration/surgical decompression of subperiosteal space if atypical presentation Surgical drainage if unresponsive to Abx Limb immobilised initially but must later mobilise to prevent deformity

Question 1151

Def: septic arthritis

Answer 1151

A serious infection of the joint space Children **\<2 years old** Usually, **monoarticular** URGENT DIAGNOSIS AND TREATMENT REQUIRED

Question 1152

Aetiology of septic arthritis

Answer 1152

Commonly, **haematogenous spread** of the pathogen Also, following a **puncture wound** or **infected skin lesion** Adjoining **osteomyelitis** Staphylococcus aureus H.influenzae in unimmunised individuals

Question 1153

Symptoms and signs of septic arthritis

Answer 1153

**Acutely febrile** child Erythematous, warm, acutely **tender joint** **Pseudoparalysis** (joint held still due to pain) **Effusion** in peripheral joint *_Septic arthritis of the hip_* difficult to diagnose due to subcutaneous fat. Initial presentation with **limp** or **pain refererred to knee**

Question 1154

Ix in septic arthritis

Answer 1154

**Acute phase reactants** – Raised **Blood cultures** **Ultrasound** – identify effusions **X-ray** – exclude trauma/other bony lesions. X-ray is NORMAL apart from joint space widening and soft tissue swelling **MRI** – identify an adjacent osteomyelitis

Question 1155

Gold standard Ix in septic arthritis?

Answer 1155

Joint space aspiration under USS with culture

Question 1156

Mx of septic arthritis

Answer 1156

Abx: prolonged course, initially IV Joint wash out or surigcal drainage if slow resolution or deep-seated joint Immobilised intially but must be mobilised later to prevent deformity

Question 1157

Def: SCFE

Answer 1157

Displacement of the epiphysis of the femoral head postero-inferiorly PROMPT treatment required to prevent **avascular necrosis** **Adolescents** at 10-15 years of age during the **adolescent growth spurt** 20% are bilateral

Question 1158

Associations of SCFE?

Answer 1158

Obesity Metabolic endocrine abnormalities: hypothyroidism, hypogonadism

Question 1159

Restricted abduction and internal rotation of the hip in an adolescent boy?

Answer 1159

SCFE

Question 1160

Ix in SCFE

Answer 1160

XR with a frog lateral view requested

Question 1161

Mx of SCFE

Answer 1161

Surgical: pin fixation in situ with prophylactic fixation of contralateral hip

Question 1162

Answer 1162

Question 1163

Limp or abnormal gait Asymmetry of skinfolds around hip Limited abduction of the hip Shortening of the affected leg

Answer 1163

DDH

Question 1164

Sensitivity and specificity of neonatal screening for DDH

Answer 1164

Per 1000 live births, 6-10 detected, 1.3 true DDH

Question 1165

Ix in DDH

Answer 1165

Neonatal screening: Barlow, Ortolani USS (gold standard) XR

Question 1166

Mx of DDH

Answer 1166

Majority resolve spontaneously Splint or harness to keep hip flexed and abducted for several months with progress monitored with USS and XR

Question 1167

Def: Perthes disease

Answer 1167

**Avascular necrosis** of the **capital femoral epiphysis** of the femoral head due to **interruption of the blood supply** Followed by **revascularisation** and **reossification** over 18-36 months **Boys** **5-10 years of age** 10-20% are bilateral

Question 1168

Cx of Perthes

Answer 1168

Femoral head deformity and metaphyseal damage leading to subsequent degenerative arthritis in adult life

Question 1169

Signs/symptoms of Perthes disease

Answer 1169

Insidious onset Limp or hip/knee pain

Question 1170

Ix in Perthes

Answer 1170

XR both hips with frog views Bone scan MRI

Question 1171

Answer 1171

Question 1172

Mx of Perthes disease

Answer 1172

If detected early and less than half of the femoral head affected: Bed rest and traction If late presentation or severe disease: Femoral head needs to be covered by acetabulum to act as mould for re-ossifying epiphysis Hip should be maintained in abduction with plaster or calipers Femoral/pelvic osteotomy also an option

Question 1173

Def: Osgood-Schlatter disease

Answer 1173

**Osteochondritis** of the **patellar tendon** insertion at the **knee** **Overuse** syndrome that affects **adolescent males** who are **physically active** (particularly basketball or football) (repeated knee flexion and forced extension)

Question 1174

Cx of Osgood-Schlatter

Answer 1174

Pain as an adult due to formation of a separate ossicle at the tibial tubercle

Question 1175

Signs/symptoms of Osgood-Schlatter

Answer 1175

Knee pain after exercise Localised tenderness Swelling over the tibial tuberosity Hamstring tightness

Question 1176

Dx of Osgood-Schlatter

Answer 1176

Clinical

Question 1177

Mx of Osgood-Schlatter?

Answer 1177

Most resolve with reduced activity NSAIDs PT: quadriceps muscle strengthening, hamstring stretches Orthotics Knee immobiliser splint Surgical mx: excision of the affected part of the tibial tubercle

Question 1178

Def: Leukaemia

Answer 1178

Uncontrolled proliferation of a lymphoid progenitor cell that is genetically altered. Early lymphoid precursors replace normal heamatopoeitic cells of the bone marrow **Acute lymphoblastic leukaemia** may present with bone pain in children (sometimes **primarily at night**)

Question 1179

Signs/symyptoms of leukaemia

Answer 1179

General: FLAWS Bone marrow infiltration: Anaemia Neutropenia Thrombocytopenia Reticulo-endothelial infiltration: Hepatosplenomegaly Lymphadenopathy Superior mediastinal obstruction CNS Testicular englargement

Question 1180

What is the commonest chronic inflammatory joint disease in children and adolescenets?

Answer 1180

JIA

Question 1181

Def: JIA

Answer 1181

**Persistent joint swelling** (\>6 weeks) presenting **before 16 years of age** in the **absence of infection or any other defined cause** 95% have a disease that is clinically and immunogenetically distinct from rheumatoid arthritis in adults 7 subtypes

Question 1182

What are the Cxs of JIA

Answer 1182

Chronic anterior uveitis Flexion contractures of the joints Growth failure Osteoporosis Amyloidiosis Constitutional problems

Question 1183

Signs/symptoms of JIA

Answer 1183

Gelling: stiffness after periods of rest Morning stiffness and pain Avoidance of previousl enjoyed activities, deteriorating behaviour/mood Joint swelling and inflammation In chronic arthritis: synoval thickening and swelling of the periarticular soft tissues Long term: bone expansion from overgrowth: Knee- leg lengthening or valgus deformity Hands: discrepant finger lengths Wrist: advancement of bone age

Question 1184

Mx of JIA

Answer 1184

NSAIDs Joint infections Methotrexate Systemic steroids Biologics MDT: specialist paeds rheum, specialist nurses, PT, opthalmology, dentristy, orthopaedics, social services OH

Question 1185

What are the subtypes of JIA

Answer 1185

Oligoarthritis (persistent) Oligoarthritis (extended) Polyarthritis (RF negative) Polyarthritis (RF positive) Systemic arthritis Psoriatic arthritis Enthesitis-related arthritis Undifferentiated arthritis

Question 1186

An 8 week old male infant is brought in by his mother to see the GP. She states that his right testis is undescended since birth. She was advised by a doctor when the child was born that she should take him to a doctor at 6 to 8 weeks of age if the problem persisted which is why she has brought him to the GP. On examination the GP confirms that there is a unilateral undescended testis on the right; the penis appears normal. What would be the next step in management? ## Footnote Review at 3 months of age Review at 6 months of age Arrange genetic and hormonal testing Arrange ultrasound scan Refer to paediatric surgeon

Answer 1186

If the testis is undescended by 3 months of age, a diagnosis of cryptorchidism can be made. At this point the child should be referred to a paediatric surgeon and seen before 6 months of age.

Question 1187

A 5-year-old boy is brought to his GP by his father complaining of abdominal pain for the last two weeks. He has not had any diarrhoea or vomiting and there has been no weight loss. He had a urinary tract infection when he was 4 years old. On abdominal examination the GP feels a mass on the right side of the abdomen. What would be the most important step in his management? Abdominal ultrasound Urgent referral for specialist assessment within two weeks Urine dipstick Urgent referral for specialist assessment within 48 hours Abdominal x-ray

Answer 1187

The key here is the abdominal mass which should trigger an immediate urgent referral to a paediatrician as this is usually how Wilms' nephroblastoma presents. The previous UTI is of no significance here. NICE advocate urgent referral if any unexplained abdominal mass in present.

Question 1188

A 2-year-old boy with meningococcal septicaemia arrests on the ward. You are the first person to attend. After confirming cardiac arrest and following paediatric BLS protocol, what is the rate you should perform chest compressions at? 140-160 compressions per minute 160-180 compressions per minute 120-140 compressions per minute 100-120 compressions per minute 80-100 compressions per minute

Answer 1188

The UK Resuscitation Council's Paediatric Basic Life Support guideline states that chest compressions for children of all ages must be performed at a rate of 100-120 per minute. Compressions should depress the sternum by at least a third of the depth of the chest. Lay persons and those not trained in paediatric resuscitation are advised to use the adult chest compression to rescue breaths ratio of 30:2, however those caring for children and trained to do so should use a ratio of 15:2.

Question 1189

A 10-year-old boy with atopic eczema is brought in to see his GP by his mother. She states that his eczema has flared up recently, with areas of itchy dry skin that are red and sometimes bleed when he scratches. This is confirmed by the GP on examination. In addition to emollients, the GP prescribes topical betamethasone valerate 0.025% to treat this moderately severe flare. For how long after the flare has been controlled should the GP advise the steroid cream be continued? 24 hours 48 hours 72 hours 1 week 2 weeks

Answer 1189

For moderate flares of atopic eczema, NICE advise using emollients generously in conjunction with a moderately potent topical steroid cream such as betamethasone valerate 0.025% or clobetasone butyrate 0.05%. Treatment should continue for 48 hours after the flare has been controlled. For delicate areas such as the face and flexures, a mildly potent steroid should be tried first (for example 1% hydrocortisone) and stronger steroids should only be used if this fails to control symptoms. A maximum of 5 days of topical steroids should be prescribed.

Question 1190

Mx of Impetigo

Answer 1190

Topical fusidic acid is first line Retapamulin is second line if fusidic acid has been ineffective if poorly tolerated Mupirocin should be used if ?MRSA In extensive disease: Oral fluclox or Oral erythromycin if penallergic

Question 1191

A 5-year-old girl is brought in to see her GP by her mother complaining of increased frequency of passing urine and dysuria. This has never happened before and she is otherwise well. The GP asks for a urine sample to be given before starting antibiotics. Pending culture results, he decides to prescribe a 3-day course of antibiotics. Which antibiotic would be most appropriate in this case? Trimethoprim Amoxicillin Cefalexin Nitrofurantoin Clarithromycin

Answer 1191

NICE CKS advise using an oral antibiotic for 3 days pending culture results. Nitrofurantoin is not licensed for a 3-day course. Amoxicillin resistance is common so it should ideally only be used if the culture and sensitivities show that the organism is sensitive. Cephalosporins should be avoided if more narrow-spectrum antibiotics would work due to the increased risk of MRSA, Clostridium difficile and resistant UTIs. For more on urinary tract infection in children see the NICE CKS guideline: http://cks.nice.org.uk/urinary-tract-infection-children#!topicsummary

Question 1192

Def: physiological jaundice

Answer 1192

Jaundiace appearing \>24h after birth, not lasting more than 2w in term and 3w in preterm infnat

Question 1193

Def: prolonged jaundice

Answer 1193

Lasting \>2w in term, 3w in preterm

Question 1194

Why are neonates more susceptible to jaundice?

Answer 1194

Shortened RBC lifespan Immature liver funciton (less glucuronyl transferase) Higher rate of Hb catabolism Innately polycythaemic

Question 1195

Epidemiology of neonatal jaundice

Answer 1195

60% of term 80% of preterm

Question 1196

Risk factors for neonatal jaundice

Answer 1196

Prematurity Jaundice \<24h (ABO, Rhesus) UDP-glucuornyl transferase deficiency (Crigler-Najar, Gilbert's) Poor feeding Infection DM mother Cephalohaematoma Polycythaemia Ethnicity

Question 1197

Cx of neonatal jaundice

Answer 1197

Unconjugated bilirubin leading to kernicterus: bilirubin encephalopathy

Question 1198

When is there an increased risk of kernicterus

Answer 1198

Serum bilirubin \>340 micromol/l in term Rapidly rising bilirubin of \>8.5 micromol/l per hour Clinical features of kernicterus

Question 1199

Symptoms of biliary atresia

Answer 1199

Pale stool, Dark urine

Question 1200

What is the most important thing to determine in neonatal jaundice?

Answer 1200

Where it is a conjugated or unconjugated bilirubinaemia

Question 1201

Causes of unconjugated bilirubinaemia

Answer 1201

Haemolytic disease: ABO, Rhesus etc. RBC abnormalities: HS, G6PD Bilirubin conjugation defects: CN, Gilbert's Sepsis Breast milk jaundice

Question 1202

Causes of conjuigated neonatal jaundice

Answer 1202

Biliary atresia Biliary obstruciton e.g. cholelithiasis, cholecystitis CF Hepatitis Birth asphyxia A1AT deficiency Haemosiderosis

Question 1203

Ix in neonatal jaundice

Answer 1203

Examination Measure bilirubin: transcutatneous bilirubinomete at \>35w gestation and \>24h after birth If this is high: serum Always measure serum in babies who are jaundice \<24h after birth and are \<35w FBC Blood film LFT Bilirubin split U&Es CRP Blood group Coomb's G6PD levels Septic screen TFT TORCH Titres A1AT Sweat chloride USS Hepatobiliary radionuclide scans Liver biopsy

Question 1204

Mx of neonatal jaundice

Answer 1204

Continue breast feeding Treat underlying cause Phototherapy to reduce conjugated bilirubin- baby needs eye protection, T monitoring, hydration Check serum bilirubin after 4-6h and every 6-12h Exchange transufion and IVIG if necessary Refer to nomogram- have lower threshold for starting therapy in preterm

Question 1205

Causes of haemolytic disease of the newborn

Answer 1205

ABO incompatibility: +ve Coomb's, spherocytes Rhesus incompatibility: maternal anti-Rh, positive Coomb's, nucleated RBC

Question 1206

Indicators that HS may be the cause of neonatal jaundice

Answer 1206

FHx AD Spherocytes Splenomegaly Positive red cell osmotic fragility test

Question 1207

Mx of HS

Answer 1207

Folic acid Splenectomy Immunisation: pneumovax, Hib, MenC Lifelong penicillin

Question 1208

Featurse of biliary atresia

Answer 1208

Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis leading to obstruction and jaundice Persistent jaudnice with pale stools and dark urine Splenomegaly not normally a feature FTT Abnormal LFTs (GGT) USS used to ddx from neonatal hepatitis

Question 1209

Mx of biliary atresia

Answer 1209

Abx to prevent cholangitis Ursodeoxycholic acid to encourage bile flow Fat-soluble vitamin supplementation Nutritional support Surgery: portoenterostomy

Question 1210

Draw the causes of neonatal jaundice

Answer 1210

Question 1211

Most common cause of nappy rash

Answer 1211

Contact dermatitis

Question 1212

Risk factors for nappy rash

Answer 1212

Infrequent nappy changing Diarrhoea Urea splitting organisms in faeaces

Question 1213

Symptoms of nappy rash

Answer 1213

Convex surfaces of the buttocks, perineal region, lower abodmen and top of thighs Sparing of flexures Erythematous rash with scalded appearance Severe forms association with erosions

Question 1214

Mx of nappy rash

Answer 1214

Protective emollient Severe: topical corticosteroids No nappy

Question 1215

Answer 1215

Nappy rash

Question 1216

Answer 1216

Infant seborrhoeic dermatitis

Question 1217

Features of infantile seborrhoeic dermatitis

Answer 1217

Eruption of unknown cause presenting around 2m Increased risk of developing atopic eczema Starts on scalp as an erythematous scaly eruption Scales form a thick yellow adherent layer: cradle cap Child not distrubed by it

Question 1218

Mx of infantile seborrhoeic dermatitis

Answer 1218

Mild emollients Scalp treated with ointment containing low concentration sulpher and salicylic acid Widespread eruptions treated with mild topical corticosteroid +/- antibacterial/fungal as appropriate

Question 1219

Answer 1219

Millia Retention of kertaine and sebaceous material in the pilaceous follicles that resolves spontaneously

Question 1220

Answer 1220

Erythema toxicum neonatorum * Common, 2-3d * White pinpoint papules at the centre of an erythematous base * Fluid contains eosinophils. * Concentrated on the trunk, come and go at different sites

Question 1221

Answer 1221

**Mongolian blue spot** * Blue/black macular discolouration at the base of spine and on the buttocks * Afro-Caribbean/ Asian * Fade slowly over 1^st few years * **Ddx: NAI**

Question 1222

Peripheral cyanosis of the hands and feet in newborn

Answer 1222

Common in the first day

Question 1223

Epstein pearls

Answer 1223

Small white pearls along the midline of the palate (resolve spontaenously)

Question 1224

Breast enlargement in newborns

Answer 1224

Resolve spontaneously May discharge milk

Question 1225

Umbilical hernia in newobrns

Answer 1225

Common, especially in afro-carribean No treatment indicated for the first 2-3y as they usually resolve spontaenously

Question 1226

Positional talipes

Answer 1226

Feet often remain in their in utero position, unlike true talipes quinovarus, the foot can be fully dorsiflexed to touch the fron of the lower leg

Question 1227

2 important causes of jittery baby

Answer 1227

Hypoglycaemia Drug withdrawal/neonatal abstinence syndrome

Question 1228

When is hypoglycaemia in neonates more common?

Answer 1228

In the first 24h in babies with: IUGR Preterm Mothers with DM Large-for-dates Hypothermic Polycythaemic Ill

Question 1229

What is the optimum target for glucose levels in baby

Answer 1229

\>2.6mmol/l

Question 1230

Aetiology of hypogylcaemia in neonates

Answer 1230

IUGR and preterm: poor glycogen stores Infants of DM have high insulin levels due to pancreatic islet hyperplasia

Question 1231

Signs/symptoms of hypoglycaemia in neonate

Answer 1231

Jitteriness Irritability Lethargy Drowsiness Seizures

Question 1232

Mx of hypoglycaemia

Answer 1232

Prevent by early and frequent milk-feeding Monitor blood glucose regulalry IV dextrose infusion if 2 readings \<2.6 or 1 \<1.6 or Symptomatic Infusion given by central venous catheter Glucaogn or hydrocortisone if there is difficulty administering infusion

Question 1233

Why should dextrose infusion for neonates be given via central venous catheter?

Answer 1233

To avoid extravasation into tissues

Question 1234

Symptoms of neonatal abstience syndrome?

Answer 1234

Jitteriness Mottling Diarrhoea Fever Hyperactive reflex Hypertonia Poor feeding Tachypnoea Seizures Sweating Tremors Vomiting Irritability

Question 1235

Ix in neonatal abstinence syndrome

Answer 1235

Neonatal abstience syndrome scoring system Toxicology screen of meconium Urinalysis

Question 1236

Mx of neonatal abstinence syndrome

Answer 1236

IV fluids Higher-calorie formula Morphine Methadone Initial addictive drug with a dose titrated down

Question 1237

Substances associated with neonatal abstinence syndrome

Answer 1237

Amphetamines, barbiturates, benzodiazepines (diazepam, clonazepam), cocaine, opiates/narcotics (heroin, methadone, codeine)

Question 1238

Def: TTNB

Answer 1238

Commonest cause of respiratory distress Diagnosis of exclusion Increased O2 requirement, RR, ABG doesn't reflect CO2 retention

Question 1239

Mx of TTN

Answer 1239

Usually settles within first day of life Additional O2 may be required

Question 1240

Features of RDS

Answer 1240

Deficiency of surfactant (secreted by Type 2 pneumocytes) Leading to widespread alveolar collapse and inadequate gas exchange Most common \<28w More severe in boys Mothers with DM- term

Question 1241

Symptoms of RDS

Answer 1241

Increased RR Laboured breathing: chest wall recession, nasal flaring Gruntin Cyanosis

Question 1242

Mx of RDS

Answer 1242

Steroids Surfactant therapy May require O2 supplemented with CPAP or artifical ventilation

Question 1243

Pneumothorax in newborn

Answer 1243

Occurs in RDS as a result of overdistended alveoli, may track into intersittium- pulmonary interstitial emphysema Also occurs in babies who are ventilated

Question 1244

Mx of pneumothorax in neonates

Answer 1244

Use lowest pressure ventilation that provide adequate chest movement and satisfactory blood gases

Question 1245

Draw the causes of respiratory distress in term infants

Answer 1245

Question 1246

Draw the classification of causes of a floppy infant

Answer 1246

Question 1247

Def: congenital muscular dystrophy

Answer 1247

Heterogeneous group of inherited disorders presenting at birth/early infancy with weakness, hypotonia or contractures Slowly progressive proximal weakness with tendency to contracture when ability to walk is lost Disease of muscle membranes or supporting proteins Biopsy shows dystrophic features with reduction of one of the ECM proteins (usually laminin)

Question 1248

Def Congenital myopathy

Answer 1248

Defined by static hisotchemical or ulstrastructural changes on muscle biopsy Caused by genetic defects in contractile apparatus of muscle Presents at birth with generalised hypotonia and muscle weakness CK usually normal or mildly elevated

Question 1249

Def: myotonia

Answer 1249

Delayed relaxation after sustained muscular contracition Hypotonia, feeding problems and respiratory difficulties Dominant inheritance caused by nucleotide triplet repeat expansion **Examine the mother for myotonia: slow release handshake or difficulty releasing a tightly clasped wrist**

Question 1250

Transient neonatal myasthenia

Answer 1250

Affects 10% of infants born to mothers with MG Symptoms appear within 72h of birth and persist for several days to 3m Respiratory and feeding difficulties Generalised hypotonia Responds well to AChE inhibitors

Question 1251

Mx of periorbital cellulitis

Answer 1251

Refer all patients Prompt IV abx- coamoxiclav

Question 1252

Features of periorbital cellulitis

Answer 1252

Inflammation of the eyelids Caused by bacterial infection (staph and strep, Hib in unimmunised), spread from sinusitis or dental abscess or as a consequence of local trauma Cx is orbital ceullitis Fever, erythema of eyelids, tenderness and warm

Question 1253

Cx of orbital cellulitis

Answer 1253

Permanent vision loss Abscess formation Meningitis Cavernous sinus thrombosis

Question 1254

Symptoms of orbital cellulitis

Answer 1254

Proptosis Painful or limited ocular movement Reduced visual acuity

Question 1255

Mx of orbital cellulitis

Answer 1255

CT head to assess posterior spread LP IV Abx: cef and fluclox Monitor optic nerve funciton every 4 hours

Question 1256

Vision at birth

Answer 1256

Face fixation and following

Question 1257

Vision at 6-8w

Answer 1257

Follows bright toy Optokinetic nystagmus

Question 1258

Vision at 6m

Answer 1258

Reaches well for toys Preferential looking

Question 1259

Vision at 2.5y

Answer 1259

Can identify or match pictures of reducing size (Kay pictures)

Question 1260

Vision at 4y

Answer 1260

Crowded LogMAR

Question 1261

Vision at 6y

Answer 1261

LogMAR

Question 1262

Def: blind child

Answer 1262

If education can only be provided by methods not involving sight Paritally sighted: education can be provided using large print books

Question 1263

What are the commonest causes of blindness?

Answer 1263

Optic atrophy Congeital cataracts Choroideoretinal degeneration

Question 1264

Answer 1264

Question 1265

What are the right to left shunts?

Answer 1265

Tetralogy of fallot TGA Eisenmenger

Question 1266

What are the Left to Right shunts?

Answer 1266

ASD VSD PDA

Question 1267

What are the common mixing congenital heart defects?

Answer 1267

AVSD COmplex coongenital heart disease

Question 1268

What are the causes of outflow obstruction?

Answer 1268

AS PS Adult-type coarctation of the aortaq

Question 1269

What is a secundum ASD?

Answer 1269

Defect in the centre of the atrial septum involving the foramen ovale Partial RBBB common

Question 1270

What are te symptoms of ASD?

Answer 1270

None/recurrent chest infections Arrythmias (later on in life)

Question 1271

Ejection systolic murmur at the upper left sternal edge Fixed and widely split second heart sound?

Answer 1271

ASD

Question 1272

Mx of Secundum ASD?

Answer 1272

Catheter device closure at 2-5y

Question 1273

Mx of partial AVSD (primum)?

Answer 1273

Surgical closure at 3 yeasr

Question 1274

What is partial AVSD?

Answer 1274

Primum Interatrial communication between the bottom end of the atrial septum and the AV valves and abnormal AV valves (apical pansystolic murmur)

Question 1275

Def: VSD

Answer 1275

Defect anywhere in hte ventricular septum, perimembranous or muscular

Question 1276

Small VSD=

Answer 1276

Smaller than the aortic valve

Question 1277

Asymptomatic loud pansystolic murmur at LLSE, quiet P2 Normal examination and ECG

Answer 1277

Small VSD

Question 1278

Mx of small VSD

Answer 1278

Spontaneous closure

Question 1279

Heart failure FTT after 1wk Recurrent chest infections Tachycardia Tachypnoeic Hepatomegaly Active precordium Soft pancystolic murmur with loud P2 Upright T wave

Answer 1279

Large VSD

Question 1280

Mx large VSD

Answer 1280

Rx for HF surgery at 3-6/12

Question 1281

Def: PDA

Answer 1281

Failure to close by 1/12

Question 1282

Continuous murmur beneath the left clavicle Murmur continuing into diastole because the pressure in the pulmonary artery is lower than in the aorta throoughout the cardiac cycel Rasied pulse pressure Collapsing pulse

Answer 1282

PDA

Question 1283

Tetralogy of Fallot

Answer 1283

Large VSD Overriding aorta Subpulmonary stenosis causing RB outflow tract obstruction RVH

Question 1284

Clubbing Loud, harsh ESM at LSE

Answer 1284

Tetralogy of Fallot

Question 1285

Symptoms of RVH

Answer 1285

Severe cyanosis Hypercyanotic spells Squatting on exercies

Question 1286

Question 1287

Mx TOF

Answer 1287

Initially medical Sx at 6/12

Question 1288

Def: TGA

Answer 1288

Aorta connected to RV and pulmonary connected to LV

Question 1289

Profound cyanosis espeically in D2 of life HS2 often loud and single Usually no murmur

Answer 1289

TGA

Question 1290

Answer 1290

Question 1291

Mx of TGA

Answer 1291

Improve mixing- maintain PDA with prostaglandin infusion Balloon atrial septostomy Sx

Question 1292

Pathogenesis of eisenmenger

Answer 1292

When L to R shunt/common mixing not treated early, pulmonary arteries become thickened This leads to resistance to flow Gradually becomes less symptomatic Eventually at 10-15y the shunt reverses and they become blue Progressive leading to death from RHF in adulthood

Question 1293

Features of complete AVSD

Answer 1293

DS Defect in the middle of the heart with a single 5 leaflet valve between the atria and the ventircles Pulmonary HTN Cyanosis at birth, HF at 2-3w

Question 1294

AS in newborn

Answer 1294

Valves partly fused together

Question 1295

Asymptomatic murmur, small volume, slow rising pulse, carotid thrill ESM at URSE radiating to neck and soft A2 Apical ejection click

Answer 1295

AS

Question 1296

ESM at USLE, ejection click

Answer 1296

PS

Question 1297

Downgoing T wave

Answer 1297

LV strain and severe AS

Question 1298

ECG in PS

Answer 1298

Upright T wave in V1= RVH

Question 1299

* Asymptomatic, systemic HTN in the R arm, ESM at USE, collaterals heard with continuous murmur at the back, radio-femoral delay * CXR: rib notching – development of large collateral intercostal arteries * 3 sign – visible notch in the descending aorta

Answer 1299

Coarctation of the aorta

Question 1300

Downgoing T wave LV strain and severe coarctation +/- HTN

Answer 1300

Coarctation

Question 1301

Answer 1301

3 sign Coarctation of the aorta

Question 1302

Sick with HF and shock in the neonatal period=

Answer 1302

Outflow obstruction in the sick infant PG ASAP and early surgical intervention

Question 1303

Causes of outflow obstruction in a sick infant

Answer 1303

Aortic coarctation Interruption of the aortic arch Hypoplastic left heart syndrom

Question 1304

Features of aortic coarctation

Answer 1304

Arterial duct tissue encricling the aorta at the point of insretion of hte duct so when the duct closes it causes constriction of the aorta Acute ciruclatory collapse at 2/7 when duct closes Absent femoral pulses Severe metabolic acidosis

Question 1305

Features of interruption of the aortic arch

Answer 1305

No connection between hte proximal aorta and the distal arterial duct CO dependant on Right to left shunt via the duct VSD present Associated with Di George Syndrome

Question 1306

Causes of HF in neonate

Answer 1306

Obstructed (duct-dependant) systemic circulation Hypoplastic left heart Crtical aortic valve stenosis Severe coarctation of the aorta Interruption of the aortic arch

Question 1307

Causes of HF in infants

Answer 1307

VSD AVSD Large PDA

Question 1308

Causes of HF in older children and adolescents

Answer 1308

Eisenmenger Rheumatic heart disease Cardiomyopathy

Question 1309

Sign of RVH in children

Answer 1309

Upright T wave in V1

Question 1310

Sign of left ventricular strain in children

Answer 1310

Inverted T wave in V6

Question 1311

Pitfalls in ECGs of children

Answer 1311

P wave morphology unhelpful Partial RBBB common, also seen in ASD Sinus arrythmia is a normal finding

Question 1312

Breathless/asymptomatic CHD

Answer 1312

Left to right

Question 1313

Blue CHD

Answer 1313

Right to left shunt

Question 1314

Breathless and blue CHD

Answer 1314

Common mixing defect e.g. AVSD or complex congenital heart disease

Question 1315

Answer 1315

Question 1316

Answer 1316

Question 1317

Answer 1317

PDA

Question 1318

Mx of preterm infants

Answer 1318

Maintain environmental temperature Non oral feeding Mx of complications

Question 1319

Cx of preterm infants

Answer 1319

Hypothermia Metabolic: hypoglycaemia, hypocalcacemia, jaundice Respirator: respiratory distress Feeding problems Intracranial haemorrhage Infection PDA Retinopathy of prematurity NEC

Question 1320

Px of preterm infnats

Answer 1320

Excellent if \>32w Viable if 24w \<1.5kg are at risk of neurodevelopmental problems

Question 1321

What are the respiratory problems of \<1.5kg infants

Answer 1321

RDS Pneumothorax Apnoea and bradycardia and desturations Bronchopulmonary dysplasia

Question 1322

What are the circulatrory problems in infants \<1.5kg

Answer 1322

Hypotension PDA

Question 1323

What are the nutritional issues in infants \<1.5kg

Answer 1323

NG tube Feeding intolerance- TPN

Question 1324

What are the metabolic problems in infants \<1.5kg

Answer 1324

Hypoglycaemia Electrolyte distrubances Osteopenia of prematuiry- from low phosphate

Question 1325

What are the brain injury considerations in infants \<1.5kg

Answer 1325

IVH Ventricular dilatation Periventricular leukomalacia

Question 1326

Answer 1326

Question 1327

Mx of PDA

Answer 1327

PG synthetase inhibitor Ibuprofen Indomethacin Surgical ligation

Question 1328

Clinical examination pneumothorax in preterm

Answer 1328

Transillumination of the chest

Question 1329

What causes apnoea, bradycardia and desaturation in preterm?

Answer 1329

Common until 32w Immaturity of central respiratory control Stops breahting for 20-30s or when breathing continues against closed glottis Starts again after physical stimulation

Question 1330

Mx of apnoea, bradycardia and desaturation

Answer 1330

CPAP and caffiene

Question 1331

Fluid requirements of preterm infant

Answer 1331

60-90ml/kg on first day Titrate according to urine output and weight change until 150-180ml/kg/d

Question 1332

Nutritional considerations in preterm infants

Answer 1332

High metabolic demands Also Fe deficient as Fe stores are transferred in third trimester.

Question 1333

Mx of nutrition in preterm infants

Answer 1333

Oro/NG tube with special feeds: phosphate, protein and calories need suppleneting Breastmilk ASP TPN can be used (PICC line)

Question 1334

Preterm brain injury & periventricular haemorrhage

Answer 1334

* 25% of very low BW infanats * In germinal matrix above caudate nucleus à fragile network of blood vessels * Large intraventricular haemorrhage à disrupt drainage and reabsorption of CSF à build up of CSF pressure à dilatation of ventricles à hydrocephalus à cranial sutures can separate à tense anterior fontanelle. Mx: ventriculoperitoneal shunt, symptomatic relief with LP or ventricular tap to release CSF and reduce pressure * Periventricular white matter brain injury (ischaemia/inflammation without haemorrhage) à cystic lesions around ventricles; if bilateral multiple cyst: periventricular leukomalacia (PVL) * CAN OCCUR WITHOUT CLINICAL SIGNS

Question 1335

Features of NEC

Answer 1335

* Bacterial invasion of the bowel wall * More likely if NOT breastfed * Stops tolerating feeds milk aspirated bile-stained vomiting distended abdo + blood-stained stools can become rapidly shocked and need ventilation can perforate * X-ray: distended bowel loops, intralmural gas, gas in portal tract * Mx: stop oral feeds, broad spectrum antibiotics to cover aerobes + anaerobes. Parenteral nutrition, ventilation and cirulatiory support considered. Surgery if perforation LT effects: strictures and malabsorption

Question 1336

Retinopathy of Prematurity

Answer 1336

* High levels of O2 from artificial ventilation affects developing vessels at the junction of the vascular and non-vascular retina * Vascualr proliferation detachment, fibrosis, blindness Mx: laser therapy

Question 1337

Def: Bronchopulmonary dysplasia

Answer 1337

Infants who still need O2 post 36w

Question 1338

Features of BPD

Answer 1338

* Defn: infants whostill need O2 at post-menstrual age of 36 wees * From pressure and volume trauma of artificial ventilation, oxygen toxicity, infexn * CXR: widespread opacification, cystic changes Severe disease à risk of infexn (RSV, pertussis), pulmonary hypertension

Question 1339

Port wine stain along distribution of trigeminal nerve with intracranial vascular anomalies

Answer 1339

Sturge-Weber

Question 1340

Port wine stain with severe lesions on limbs and bone hypertrophy

Answer 1340

Klippel-Trenaunay syndrome

Question 1341

Answer 1341

Klippel Trenaunay Syndrome

Question 1342

Answer 1342

Port Wine Stain

Question 1343

Answer 1343

Sturge Weber

Question 1344

Def: FTT

Answer 1344

Suboptimal weight gain in infants and toddlers

Question 1345

Mild FTT

Answer 1345

Fall across 2 centile lines

Question 1346

Severe FTT

Answer 1346

Fall across 3 centile lines

Question 1347

Weight below 0.4th centile

Answer 1347

Always trigger an evaluation

Question 1348

Important components of history in FTT

Answer 1348

Detailed dietary hx: food diary: Inadequate availabiltiy of food, psychosocial deprivaiton, neglect, food intolerance Feeding: details of what happens at meal times Childs general health i.e. chronic illness Premature, IUGR, PMH Growth of other family members (constitutional delay), autoimmune Normal development Problems at home?

Question 1349

Mx of faltering growth

Answer 1349

MDT Health visitor GP Dietician

Question 1350

Draw causes of FTT

Answer 1350

Question 1351

Marasmus

Answer 1351

Severe protein-energy malnutrition Weight for height \>3sd Wasting no oedema

Question 1352

Kwashiorkor

Answer 1352

Severe prtoein-energy malnutrition presenting with general oedema

Question 1353

Answer 1353

Kwashiorkor

Question 1354

Answer 1354

Marasmus

Question 1355

Complications of malnutrition

Answer 1355

Immune disorder Delayed wound healing Worse outcome in illness Permanent delay in intellectual development

Question 1356

Flaky paint skin rash with hyperkeratosis and desquamation Distended abdomen and hepatomegaly Angular stomatitis Sparse and depigmented hair Hypothermia Bradycardia Hypotension

Answer 1356

Kwashiorkor

Question 1357

Ix in malnutrition

Answer 1357

Food diary Anthropometry Lab: physiological adaptations to malnutrition

Question 1358

Mx of malnutrition

Answer 1358

Intensive nutritional support Parenteral or enteral (if GIT functioning)

Question 1359

Risk factors for DD

Answer 1359

Aerobic gram positive rod with branches Seen in relatively immunocompromised patients

Question 1360

Ping pong ball sensation of the skull

Answer 1360

craniotabes Vit DD

Question 1361

Palpable costochondral junctions

Answer 1361

rachitic rosary Vit DD

Question 1362

Harrison sulcus

Answer 1362

horizontal depression corresponding to attachment of the softened ribs Vit DD

Question 1363

Symptoms of hypocalacemia

Answer 1363

Seizures Tetany Apnoea Stridor

Question 1364

Mx of VitDD

Answer 1364

Correction of predisposing RF, daily VitD Healing occurs in 2-4weeks Monitoring

Question 1365

Non-organic causes of FTT

Answer 1365

* Feeding problems – insufficient breast milk or poor technique, incorrect prep of formula * Insufficient/unsuitable food offered * Irregular feeding times * Infant difficult to feed/ disinterested * Conflict over feeding, intolerance of normal feeding behaviour * Problems with budgeting, shopping, cooking, famine * Low SES * Psychosocial Deprivation – poor maternal-infant interaction, maternal depression, poor maternal education Neglect/child abuse- inc. factitious illness: deliberate underfeeding to generate FTT

Question 1366

Cx of non-organic FTT

Answer 1366

Children with non-organic FTT continue to under-eat A lasting deficit: remain underweight Impairment of development only Short term

Question 1367

Mx of non-organic FTT

Answer 1367

Health visitor: assess eating behaviour and provide support Paediatric dietician to assess quantity and quality of food intake SALT: specialist skills with feeding disorders Clinical psychologist Social services Nursery

Question 1368

When is active refeeding used?

Answer 1368

\<6m old Severe FTT: active refeeding

Question 1369

Risk factors for child abuse/neglect

Answer 1369

Child: failure to meet parental expectations (disability), result of forced, coercive, commercial sex Parent: MH problems, indifference, intolerance, over-anxiousness, EtOH, drug abuse Family: step-parents, domestic vioelcne, multiple/closely spaced births, social isolation, young parents Environment: poverty, unsavoury neighbourhood

Question 1370

Presentation of children with neglect

Answer 1370

Ravenously hungry Dirty Wearing inadequate clothing

Question 1371

?neglect in babies

Answer 1371

Apathetic Delayed development Non-demanding

Question 1372

?neglect in toddlers

Answer 1372

Violent Apathetic Fearful

Question 1373

?neglect in school children

Answer 1373

Wetting Soiling Relationship difficulties Non-attendence Anti-social behaviour

Question 1374

?neglect in adolescents?

Answer 1374

Self-harm Depression Oppositional, aggressive and delinquent behaviour

Question 1375

What factors should be considered in child abuse/neglect

Answer 1375

Childs age and stage of development History given by child Plausibility of the explanation Background- repeated A&E attendance, lack of medical care Delay in reporting Inconsistent histories Inappropriate reaction of parents – vague, evasive, unconcerned or excessively distressed/aggressive

Question 1376

Ix in child abuse

Answer 1376

XR: full radiographic skeletal survey with oblique views of ribs

Question 1377

Mx of child abuse

Answer 1377

Meticulous note taking Any injuries/medical findings noted and photographed Note interactions between parent and child ?safety of other siblings Inform senior members of team, paediatric radiologists, paediatric surgeons. Social services, police, teachers, lawyers

Question 1378

Symptoms of coeliac

Answer 1378

Malabsorptive syndrome at 9-24months after induction of wheat containing foods Mild, non-specific GI symptoms Anaemia FTT Abdo distension Buttock wasting Abnormal stools General irritability

Question 1379

Draw te clinical presentations of primary immune deficiency

Answer 1379

Question 1380

What are the secondary causes of immunodeficiency?

Answer 1380

HIV Malignancy Malnutrition Immunosuppression

Question 1381

Wiskot Aldrich

Answer 1381

X linked Triad of: T cell defect, thrombocytopenia and eczema

Question 1382

Duncan syndrome

Answer 1382

Inability to make a normal resposne to EBV. Either succumb to initial infection or devlop lymphoma X linked lymphoproliferative disesae

Question 1383

Ataxia telangectasis

Answer 1383

Defect in DNA repair increased risk of lymphoma Cerebellar ataxia T cell defects

Question 1384

Bruton agammaglobulinaemia

Answer 1384

Abnormal RTK essential for B cell maturation

Question 1385

CVID

Answer 1385

B cell deficiency High risk of autoimmune disorders and malignancy Later onset than Bruton

Question 1386

Delayed separation of umbilical cord and immunodeficiency

Answer 1386

Leucocyte adhesion deficiency

Question 1387

Ix in FTT

Answer 1387

FBC U+Es, ABG, ca etc LFTs TFTs Acute phase reactants Ferritin Immunoglobins IgA TTG Urine and Stool MC+S Karyotype CXR and sweat test

Question 1388

What are the dietary strategies for increasing energy intake

Answer 1388

Three meals and two snacks Increase number and variety of foods offered Increase energy density of usual foods Decrease fluid intake, particularly quash

Question 1389

What are the behavioural strategies for increasing energy intake

Answer 1389

Regular meals with family Praise when food eaten Encourage child to eat but avoid conflict

Question 1390

What are hte components of a nutritional assessment

Answer 1390

Anthropometry Laboratory: albumin, specific minerals and vitamins Food intake: diary and recall Immunodeficiency: lymphocyte count, impaired cell-mediated immunity

Question 1391

Components of anhtropometry

Answer 1391

Weight Height Mid-arm circumference Skinfold thickness

Question 1392

What are the signs of constitutional growth falling

Answer 1392

Steady grwoth below centiles or catch down for larger baby Short parents Normal physical examination

Question 1393

What are the signs of psychosocial growth faltering

Answer 1393

Crossing down of ceniles Eating difficulties, maternal depression Normal physical examination. Abnormal maternal-infant interaction

Question 1394

Answer 1394

Question 1395

Answer 1395

Question 1396

Answer 1396

Answer 1397

Question 1398

Macule

Answer 1398

Flat, circumscribed skin discolouration. Not raised or depressed Flat naevus Freckle

Question 1399

Patch

Answer 1399

Macule more than 1 cm Port wine stain, vitiligo, cafe´ au lait patch

Question 1400

Papule

Answer 1400

Circumscribed, elevated non-vesicular, non-pustular, less than 1 cm Molluscum, lichen planus

Question 1401

Plaque

Answer 1401

Broad elevated disc shape more than 1 cm

Question 1402

Nodule

Answer 1402

Circumscribed, elevated, solid Involves dermis Neurofibroma Nodular scabies

Question 1403

Wheal

Answer 1403

Local, superficial, transient oedema Urticaria

Question 1404

Vesicle

Answer 1404

Elevated, fluid filled \<0.5cm Herpes simplex virus, chicken pox

Question 1405

Bulla

Answer 1405

Vesicle \>0.5cm Bullous pemphigoid Epidermolysis bullosa Bullous impetigo

Question 1406

Pustule

Answer 1406

Circumscribed lesion containing pus

Question 1407

Erythematous

Answer 1407

Redness due to increased skin perfusion blanching

Question 1408

Purpura

Answer 1408

Red-purple non blanching due to extravasation of red cells

Question 1409

Petechiae

Answer 1409

Purpura \<2mm

Question 1410

Crust

Answer 1410

Collection of debris, dried serum and blood Impetigo

Question 1411

Erosion

Answer 1411

Partial focal loss of epidermis; heals without scarring

Question 1412

Scale

Answer 1412

Thick stratum , hyperproliferation Tinea corporis

Question 1413

Ulcer

Answer 1413

Full thickness, focal loss of epidermis and dermis; heals with scarring

Question 1414

Desquamation

Answer 1414

Peeling skin Kawasaki Scarlet fever

Question 1415

Causes of exanthematous rash

Answer 1415

Measles Rubella Parvovirus B19 VZV GAS HSV6

Question 1416

Spread of measles

Answer 1416

Coughing or sneezing or close contact with infected

Question 1417

Dx SSPE

Answer 1417

Finding high levels of measles Ab in blood and CSF

Question 1418

Clinical course of measles

Answer 1418

Prodromal phase- 2-4 days * Infects respiratory epithelium of nasopharynx. Prodromal symptoms include cough, coryza, conjunctivitis with fever * Infectious from prodromal phase to 4 days after rash appearing Exanthematous phase- a maculopapular rash, 5-7days after initial infection Koplik's spots

Question 1419

DDx measles

Answer 1419

Parvovirus B19- slapped cheek syndrome Streptococcal infection similar appearance to measles, but sore throat most prominent symptom Herpes virus type 6 (roseola infantum)

Question 1420

Dx of measles

Answer 1420

IgM in saliva or blood

Question 1421

Measles school exclusion

Answer 1421

Keep away from school for 4 days after developmen t of rash

Question 1422

Mx of measles

Answer 1422

Parvovirus B19- slapped cheek syndrome Streptococcal infection similar appearance to measles, but sore throat most prominent symptom Herpes virus type 6 (roseola infantum)

Question 1423

Group A strep=

Answer 1423

Strep pyogenes

Question 1424

Cx of GAS infeciton

Answer 1424

Are rare but include * Otitis media * Throat abscess * Sinusitis mastoiditis * Meningitis and brain abscess * Endocarditis, osteomyelitis * Liver abscess Autoimmune complications can occur later * Acute rheumatic fever * Streptococcal glomerulonephritis (2 weeks after) haematuria, decreased UO, oedema

Question 1425

Incubation period (2-4 days) Fever \>38, abdominal pain, vomiting, sore throat Exanthamatous phase- sand paper like diffuse rash on head and neck, desquamation after 1 week Strawberry tongue May have exudative tonsillitis, uvular oedema Presence of red flat spots (macules) dotted over the hard and soft palate (Forchheimer spots).

Answer 1425

Strep pyogenes (GAS)

Question 1426

Mx of GAS infection

Answer 1426

Pencillin QDS for 10d prevents development of rheumatic fever but not glomerulonephritis Paracetamol and ibuprofen Exclude from school 1d after starting Abx Return if symptoms worsen or not improved after 7d Notify HPU

Question 1427

Cx of Rubella infection

Answer 1427

* Arthritis or arthralgia * Transient thrombocytopenia- more commonly in children * Post-infectious enceph.

Question 1428

Rash- maculopapular rash starting behind ears typically then spreading to face and whole body Suboccipital and postauricular lymphadenopathy common 60% of women may get arthralgia

Answer 1428

Rubella

Question 1429

Mx of rubella

Answer 1429

Advise resolve within 5 days, lymphad may last a week Rest and fluids Avoid pregnant ladies Contact HPU Immunocomp- are not at risk so don’t need to be admitted

Question 1430

Prodrome: fever, lethargy, headache, coryza Exanthematous phase: bright red macules with slapped cheek appearance on face

Answer 1430

Parvovirus B19

Question 1431

Def: exanthem

Answer 1431

Rash that blooms towards the end of the incubation period

Question 1432

Prdrome: fever \>41 lasting for 4 days Exanthematous phase: rose-coloured maculopapular rash Vomiting, diarrhoea, cervical lymphad

Answer 1432

HSV6

Question 1433

Cx of VZV

Answer 1433

Neonatses Have an increased risk of disseminated or haemorrhagic varicella In children: Skin bacterial superinfection- eg necrotizing fasciitis- from scratching Pregnancy- can get fetal varicella syndrome Leads hypoplasis of limbs, eye defects, neurological abnormalities 1-2% risk

Question 1434

Prodrome: nausea, myalgia, headache, fever Characterised by itchy vesicular rash- starts on head and trunk, then rest of body Papulesàvesiclesàcrusting

Answer 1434

VZV

Question 1435

Mx of VZV infection

Answer 1435

Symptomatic treatment of fever and itching in a healthy child Topical calamine lotion coats scars Advice- Cut nails to prevent scratching Wear smooth cotton fabrics Adequate fluid If develops high temp tenderness around original chicken pox then come back as can be a superinfection IV acyclovir Acyclovir in healthy children is not recommended In immunocompromised children or in healthy children with varicella pneumonia or encephalitis VSZ IG Indicicated in high risk individuals Given within 10 days, protection for at approx. 3 weeks IM never IV * Immunocompromised children and adults * Newborns of mothers with varicella shortly before or after delivery * Premature infants or \<1 year * Adults without evidence of immunity * Pregnant women Pregnant woman Give acyclovir in primary care only if consented and advice from specialist Refer to hospital if fever persists, or any chest or neurological symptoms- drowsiness, headache If baby is born to an immune mother then fine as has antibodies Immunocomp Aciclovir VSZ-IG

Question 1436

Def: Acne

Answer 1436

A chronic skin condition in which blockage or inflammation of the hair follicles and accompanying sebaceous glands (known as pilosebaceous units) occurs Principally affects the face (99% of people), the back (60%), and the chest (15%), and usually first occurs around puberty Can present as inflammatory or non-inflammatory lesions Non-inflammatory: * Comedomes- black head (open) and white heads (closed) Inflammatory * Superficial plaques and pustules, cysts

Question 1437

Features of non-inflammatory acne

Answer 1437

Comedomes- black heads (open), white heads (closed

Question 1438

Features of inflammatory acne

Answer 1438

Superficial plaques and pustules, cysts

Question 1439

What bacteria colonise comedones?

Answer 1439

Propionibacterium acnes and to a lesser extent P. granulosum

Question 1440

Mild treatment of acne

Answer 1440

Topical treatment: benzoyl peroxide Topical abx or topical retinoids

Question 1441

Mx of moderate acne

Answer 1441

Oral abx- tetracycline

Question 1442

Mx of severe acne

Answer 1442

Oral retinoid- isotretinoin

Question 1443

Vernix Caseosa

Answer 1443

- covers the skin at birth - made up of water, protein and lipids - protects the skin in utero from amniotic fluid - shedding coincides with maturation of transepidermal layer

Question 1444

Answer 1444

Vernix caseosa

Question 1445

Def: bullous impetigo

Answer 1445

Uncommon blistering form of impetigo, superficial infection

Question 1446

Aetiology of bullous impetigo

Answer 1446

Staph aureus producing exfoliating toxin Toxin cleaves at desmoglein1 which join the keratinocytes to the superficial epidermis

Question 1447

Risk factors for bullous impetigo

Answer 1447

Infants Atopic eczema

Question 1448

Answer 1448

Bullous impetigo

Question 1449

Mx of bullous impetigo

Answer 1449

Cleansing and removal of crusts Wet dressings Systemic antibiotics- fluclox, erithromycin

Question 1450

Cx of bullous impetigo

Answer 1450

Can lead to large areas of superficial skin loss- dehydration If infection is systemic can lead to SSS (10%)- scalded skin syndrome!- treatment as if burns

Question 1451

Def: epidermolysis bullosa

Answer 1451

Group of genetic conditions characterised by increased skin fragility Associated with blistering of the skin and mucous membranes

Question 1452

Answer 1452

Epidermolysis bullosa

Question 1453

Aetiology of epidermolysis bullosa

Answer 1453

Mutations result in either abnormal, absent or significantly reduced levels of a specific protein that is important in epidermis to dermis adhesion, and the result is shearing of the skin, or blistering

Question 1454

How is epidermolysis bullosa classified?

Answer 1454

Based on the level of skin affected: Simplex: within epidermis Juncitonal: lamina lucida Dystrophic: cleave in dermis Kindler syndrome: mixed type

Question 1455

Symptoms of epidermolysis bullosa

Answer 1455

Blisters on the skin Occur spontaneously or following minor trauma The newborn infant with EB may present with localized or widespread blistering at birth, or within the first few days of life, Not possible to determine EB type by clinical examination. EB should be one, among many other diagnostic considerations, when evaluating a newborn with blisters and/or erosions as can be fatal

Question 1456

Dx of epidermolysis bullosa

Answer 1456

If EB is suspected, a skin biopsy should be taken from the edge of a fresh blister (1/2 blister and 1/2 uninvolved skin) that is \<12 h old IFM, antibodies conjugated with fluorochromes (rhodamine or fluorescein) are applied to skin sections and examined using ultraviolet light microscopy shows level affected

Question 1457

Mx of epidermolysis bullosa

Answer 1457

Prevent skin trauma Meticulous wound care Good nutrition Surveillance for extracutaenous complications

Question 1458

Answer 1458

Collodion baby

Question 1459

Aetiology of collodion baby

Answer 1459

X linked recessive

Question 1460

Cx of collodion baby

Answer 1460

Respiratory distress (secondary to chest restriction) Infections fluid loss electrolyte imbalances temperature instability Infants use up a lot of calories shedding and rebuilding skin so need extra feeding

Question 1461

Symptoms of collodion baby

Answer 1461

Infants are born with a parchment like membrane, shiney and tight like cling film Tightness can deform features and restrict movement This membrane thensheds over days-weeks Sometimes rarely there is normal skin underneath but majority have dry skin underneath the shed membrane- ichthyotic skin

Question 1462

Def: serborrhoeic dermatitsi

Answer 1462

Eruption of unknown cause presenting in the first two months of life

Question 1463

Leiner's disease

Answer 1463

Severe generalised seborrhoeic dermatitis: child becomes unwell with diarrhoea, vomiting and anaemia

Question 1464

Cradle cap

Answer 1464

Start of seborrhoeic dermatitis

Question 1465

Mx of infantile seborrhoeic dermatitis

Answer 1465

Tends to resolve over the first 6-12m Regular washing Baby oil on scalp Emollionts IMidazole can be prescribed if these fail Avoid steroids

Question 1466

When should food allergy be suspected?

Answer 1466

Food allergy should be suspected in children with atopic eczema particularly if associated with gut dysmotility (colic, vomiting, altered bowel habit) or failure to thrive

Question 1467

Ithcy rash involving face, scalp and extensor surfaces Nappy area usually spared Dry skin Lichenification

Answer 1467

Eczema

Question 1468

How can the severity of eczema be assessed?

Answer 1468

Visual analogue scales (0-10) capturing the child/parent/carer's assessment of severity, itch and sleep loss over the previous three days and nights. Patient-oriented Eczema Measure (POEM). Children's Dermatology Life Quality Index (CDLQI). Infants' Dermatitis Quality of Life Index (IDQOL). Dermatitis Family Impact (DFI) Questionnaire.

Question 1469

Severity of eczema (physical): Clear

Answer 1469

Normal skin, no evidence of atopic eczema

Question 1470

Severity of eczema (physical): Mild

Answer 1470

Areas of dry skin Infrequent itching

Question 1471

Severity of eczema (physical): Moderate

Answer 1471

Areas of dry skin, frequent itching, redness

Question 1472

Severity of eczema (physical): Severe

Answer 1472

Widespread areas of dry skin Incessant itching Redness

Question 1473

Severity of eczema (QoL): None

Answer 1473

No impact

Question 1474

Severity of eczema (QoL): Mild

Answer 1474

Little impact on everyday activites

Question 1475

Severity of eczema (QoL): Moderate

Answer 1475

Moderate impact on everyday activities and psychosocial wellbeing Frequently disturbed sleep

Question 1476

Severity of eczema (QoL): Severe

Answer 1476

Severe limitation of everyday activties and psychoscoial functioning Nightly loss of sleep

Question 1477

What are the potential trigger factors for eczema

Answer 1477

irritants, for example soaps and detergents (including shampoos, bubble baths, shower gels and washing-up liquids) skin infections contact allergens food allergens inhalant allergens.

Question 1478

Treatment of mild atopic eczema

Answer 1478

Emollients Mild ptoency topical corticosteroids

Question 1479

Treatment of moderate atopic eczema

Answer 1479

Emollients Modreate potency topical corticosteroids Topical calcineurin inhibitors Bandages

Question 1480

Treatment of severe atopic eczema

Answer 1480

Emollients Potent topical corticosteroids Topical calcineruin inhibitors Bandages Phototherapy Systemic therapy

Question 1481

What are the approaches to treatment of eczema

Answer 1481

Consider non-sedating antihistamines if itch Step down approach: decrease potency of steroids until lowest possible potency Start with milder steroids on the face Do not prescribe very potent steroids without dermatological advice Always ?infection

Question 1482

Symptoms of NAI

Answer 1482

Look for concerning interactions between child and parent Fractures in a non-mobile child Basal skull fracture- retinal haemorrhages Bruises- * shape of a hand * around wrist or hand –ligature marks * on the buttocks * shape of a bite Burns- cigarette, glove or stocking burn consistent with forced immersion Vague history

Question 1483

Ix in NAI

Answer 1483

Full skeletal survey with oblique rib view CT head and MRI Coag screen Opthalmologist to exclude retinal haemorrhages

Question 1484

Mx in NAI

Answer 1484

Admit child Consider siblings and alert social services Senior help Health visitors GP Police

Question 1485

Causes of erythema mutliforme

Answer 1485

* EM tends to be due to infections such as **HSV, Mycoplasma pneumonia** commonly (other infections too) but can be due to drugs SJ and TEN- tend to be due to a drug reaction

Question 1486

Symptoms of EM

Answer 1486

* no prodrome * a mild upper respiratory tract infection * rash starts abruptly, usually within 3 days * starts on the extremities, being symmetrical and spreading centrally

Question 1487

Mx of erythema multiforme

Answer 1487

Most are self-limiting Infection Withdraw any durgs that are causing it

Question 1488

SCORTEN=

Answer 1488

Used to assess Px of SJS and TEN \>3 admit to ICU ABCD, treat as burns

Question 1489

Causes of erythema nodosum

Answer 1489

Ass underlying conditions:‑ * Strep infection * Primary tuberculosis * IBD * Drug reaction Sarcoidosis

Question 1490

Symptoms of erythema nodosum

Answer 1490

* Eruptive phase- fever, aching and arthralgia * Painful rash usually appears within a couple of days * Lesions begin as red, tender nodules with borders poorly defined and they are 2 to 6 cms in diameter * Then turn bluish like a bruise Lesions then become more like an abscess

Question 1491

Mx of erythema nodosum

Answer 1491

Most cases are self-limiting Symptomatic management Cool compresses may help NSAIDs Conisder oral KI in more difficult cases

Question 1492

Sever itching occurs 2-6 weeks after infestation Worse at night Very itchy, burrows can be seen

Answer 1492

Scabies Sarcoptes scabiei

Question 1493

Distribution of scabies in older children

Answer 1493

* Between fingers and toes * Wrists (flexor part) * Axillae * Belt line * Around penis nipples and buttocks

Question 1494

Distribution of scabies in young children

Answer 1494

Palms Soles Trunks

Question 1495

Treatment of scabies

Answer 1495

he whole family should be treated regardless of symptoms **Premithrin cream (5%)**- applied to all areas below the neck and washe off after 8-12 hrs For babaies face and scalp should be included **Benzyl benzoate-** (25%) applied below neck and left on for 12 hra **Malathion lotion** -(0.5% aqueous) also effective, kept on for 12hrs

Question 1496

Tinea capitis

Answer 1496

Fungal infection of the scalp

Question 1497

Tines pedis

Answer 1497

Fungal infection of the feet

Question 1498

Ix in ringworm

Answer 1498

Woods' light: shows bright greenish yellow fluorescence

Question 1499

Mx of ringworm

Answer 1499

Topical antifungals More severe= systemic antifungal for several weeks

Question 1500

Mx of DSH

Answer 1500

Assess risk of depression Early referral if evidence of depression or self harm ideation

Question 1501

Mx of mild depression

Answer 1501

Watchful waiting (if child does not want intervention) After 4w of watchful waiting: ?individual non-directive supportive therapy, CBT, GSH **do not use antidepressants** If CBT/GSH not working after 2-3m refer to CAMHS

Question 1502

Mx of moderate to severe depression

Answer 1502

Refer to CAMHS Offer psychological therapy as first line If unresponsive after 4-6 sessions Consider MDT review and alternative therapy Consider fluoxetine catuiosly

Question 1503

Mx of resitant depression

Answer 1503

?Alternative psychotherapy If fluoxetine ineffetctive or not tolerated: sertarline or citalopram If psychotic dpression: atypical antipsychotic

Question 1504

Mx of a child/young persion at high risk of suicid etc.

Answer 1504

Consider inpatient treatment ECT if very severe depression and life threatening symptoms

Question 1505

Def: hypersensitivity

Answer 1505

objectively reproducible symptoms or signs following exposure to a defined stimulus at a dose tolerated by normal people

Question 1506

Def: allergy

Answer 1506

hypersensitivity reaction mediated by immunological mechanisms – can be IgE or non-IgE

Question 1507

Def: atopy

Answer 1507

personal/familial tendency to produce IgE antibodies to ordinary exposures. Strong association with asthma, AR, eczema, food allergy

Question 1508

Prevention of allergy

Answer 1508

exclusive breastfeeding for 3-4m, use probiotics for eczema in infancy, altering allergen exposure, prebiotics (non-digestible oligosaccharides in breastmilk), nutritional supplmenets (omega3 fatty acids, vit D, antioxidants), medication (antihistamines, immunotherapy)

Question 1509

Non-IgE mediated allergic reaction

Answer 1509

Delayed onset with varied clinical picture

Question 1510

Mx of allergy

Answer 1510

Monitor growth Symptomatic treatment with antihistamines and creams Advice on allergen avoidence Specific allergen immunotherapy e.g. SLIT

Question 1511

Def: food intolerance/hypersensitivity

Answer 1511

objectively reproducible symptoms or signs following exposure to a defined stimulus at a dose tolerated by normal people

Question 1512

Def: food allergy

Answer 1512

hypersensitivity reaction mediated by immunological mechanisms – can be IgE or non-IgE

Question 1513

Def: food aversion

Answer 1513

refuses food for psychological/behavioural reasons (ed ASD kids struggle with food of different textures)

Question 1514

Features of secondary allergy

Answer 1514

kid initially tolerant then develops allergy –due to cross-reactivity btwn proteins in frut/veg/nut amd those in the pollens they are allergic to. (oral allergy syndrome)

Question 1515

Temporary lactose intolerance (non-allergic food hypersensitvity)

Answer 1515

previously well kid develops D & V, vomiting settles but watery stool continues for a while. As the stomach recovers, temporarily unable to digest lactose.

Question 1516

Associations between allergic rhinoconjuncitivits

Answer 1516

Associations: eczema, asthma, sinusitis, adenoidal hyperthrophy If left untreated can à asthma (reactive airway disease)

Question 1517

Coryza, conjunctivitis, cough-variant rhinitis due to post-nasal dirp, or impaired daytime behaviour/concentration from sleep disturbance due to chronic blocked nose. Mouth breathing, cough, halitosis.

Answer 1517

Allergic rhinoconjunctivitis

Question 1518

nasal polyps, deviated/perforated nasal septum, mucosal swelling, depressed nasal bridge/widened bridge, horizontal nasal crease across dorsum

Answer 1518

Allergic rhinoconjunctivits

Question 1519

Answer 1519

Alergic salute/horizontal nasal crease Allergic rhinoconjuncitivits

Question 1520

Mx of allergic rhinoconjuncitvitis?

Answer 1520

Advice about allergen avoidance 1st line: * oral antihistamine (certizidine or loratadine) or * intranasal azelastine (H2 antagonist) For control whilst awaiting /predominantly nasal symptoms: * intranasal corticosteroid If poorly controlled * If on Oral antihistamine + intranasal corticosteroid * If on intranasal corticosteroid, check technique, increase dose If on highest dose of corticosteroid and symptoms persist: * add intranasal ipratropium bormide * Consider nasal decongestants Avoid systemic therapy where possible.

Question 1521

Asthma response to treatment

Answer 1521

Responidng: Continue bronchodilators 1-4h PRN. Discharge when stable on 4h treatment Continue oral predinosolone for up to 3d Not responding: Transfer to HDU and consider CXR, IV salbuatoml or aminophyllines (caution if already receiving theophyllines) Consider IV MgSO4 At discharge: Review medication and inhaler technique Provide personalised asthma action plan

Question 1522

Delivery of asthma medication aged 0-4

Answer 1522

MDI and valved spacer with face mask Nebuliser for acute episodes

Question 1523

Delivery of asthma therapy 5-8y/o

Answer 1523

MDI with vlaved spacer with mouthpiece Dry powder inhaler for reliver for mild symptoms

Question 1524

Delivery of asthma therapy 8-12y/o

Answer 1524

Consider dry powder inhalers for prevent are reliver

Question 1525

Delivery of asthma medication \>12y/o

Answer 1525

Consider breath activated MDI

Question 1526

Def: SJS

Answer 1526

Immune-complex mediated hypersensitivity disorder ranging from mild skin and mucous membrane lesions to a severe, sometimes fatal systemic illness. Erythema multiforme was previously considered a milder form of SJS without mucosal involvement but is now accepted as consensus to be a separate disorder.

Question 1527

What drugs are implicated in SJS?

Answer 1527

Allopurinol Carbamazepine Sulhponamides: trimethoprin, sulfasalazine Antivirals Anticonvulsants NSAIDs Aspirin Sertraline

Question 1528

What infections are implicated in SJS

Answer 1528

viral- HSV, EBV, coksackie, influenza, hep, variola; bacterial – group A strep, diphtheria, brucella, mycobacteria, typhoid, fungal, protozola- malaria, trichomonas

Question 1529

Immunisations associated with SJS

Answer 1529

Measles HepB

Question 1530

Non-specific URTI associated with fever, sore throat, chills, headace, arthralgia, malaise Mucocutaneous lesions develop suddenly and in clsuters of outbreaks over 2-4 weeks, not usually pruritic Severe oromucosal ulceration Resp – cough + thick purulent sputum Occular: painful red eye, purulent conjuncitivitis, photophobia,blepharitis(inflammation of eyelids) Skin: lesions commonly at sole, palm, dorsum of hand, extensor surface. Rash may be confined to trunk. Begin as macules papules, vesicles, bullae, urticarial plaques or confluent erythema. Centre of lesions: vesicular, purpuric or necrotic. Target lesions are pathognomonic\> Bullous lesions can rupture secondary infexn. Nikolsky sign positive( mechanical pressure to skin blistering) Mucosa: erythema, oedema, sloughing, blistering, ulceration, necrosis Genital: erosive vulvovaginitis or balanitis

Answer 1530

SJS

Question 1531

Answer 1531

Question 1532

How to manage child with conductive hearing loss?

Answer 1532

Correct by placing grommets

Question 1533

How to manage child with sensorineural hearing loss

Answer 1533

Ensure child has a means fo communications (e.g. sign language) Maximise hearing through the use of a hearing age Ensure appropriate schooling support provided

Question 1534

What are the risk factors for a child having hearing impairment?

Answer 1534

Severe prematurity Hx of meningitis, Hx of recurrent otitis media Significantly delayed or unclear speech Fhx of deafness Parental suspicion of deafness Child with CP Child with cleft palate Child with absent or derformed ears

Question 1535

Draw the causes of deafness in school children

Answer 1535

Question 1536

What are the two techniques for screening of neonatal hearing?

Answer 1536

Evoked otoacoustic emission Automated auditory brainstem response

Question 1537

How does EOAE work

Answer 1537

Click generated from earphones Detects normal sound vibrations from outer hair cells in the cochlea

Question 1538

Disadvantages of EOAE

Answer 1538

Misses auditory neuropathy as nerve/brainstem function not tested High false-positive rate Not a hearing test but a test of ochlear function

Question 1539

How does AABR work?

Answer 1539

Auditory stimulus provided via earphones Singal via ear and auditory nerve to brain EEG waveforms detected and analysed for normality

Question 1540

Disadvantages of AABR

Answer 1540

Affected by movement Complex computerised equipment

Question 1541

Def: acute otitis media

Answer 1541

Acute infection of the middle ear characterized middle ear inflammation A continuum of disease: Acute otitis media to otitis media with effusion (recurrent acute infection)

Question 1542

Def: recurrent AOM

Answer 1542

\>3 episodes of AOM in 6m or \>4 in a year with an absence of middle ear disease between episodes

Question 1543

Viral causes of otitis media

Answer 1543

RSV Rhinovirus

Question 1544

Bacterial causes of otitis media

Answer 1544

Pneumococcus H. influenza Morazella catarrhalis

Question 1545

Why are children at greater risk of otitis media

Answer 1545

Eustachian tubes are short and horizontal

Question 1546

Cx of acute otitis media

Answer 1546

Recurrence Perforation and otorrhoea- chronic supparative OM Mastoiditis Meningitis (bottom two rare nowadays)

Question 1547

haemorrhagic bullae (blisters) on the tympanic membrane

Answer 1547

= Bullous myringitis (caused by M pneumoniae, spontaneously resolves)

Question 1548

Answer 1548

Bullous myringitis Caused by M pneumoniae

Question 1549

Answer 1549

Normal tympanic membrane

Question 1550

Answer 1550

Otitis media

Question 1551

Mx of otitis media

Answer 1551

Analgesia: regular rather than PRN Abx: delay prescribin Ask them to use if symptoms persist after 4d or child getting worse

Question 1552

Rx in otitis media

Answer 1552

5d amoxicllin | (clarithromycin if pen allergic)

Question 1553

When to refer a child with otitis media

Answer 1553

\<3m old with \>38c Child 3-6m old with \>39 Recurrent OM causing effusion

Question 1554

Def: otitis media with effusion

Answer 1554

* Characterised by collection of fluid within the middle ear without any inflammation signs Can cause conductive hearing impairment

Question 1555

Answer 1555

Otitis media with effusion

Question 1556

Aetiology of OM with effusion

Answer 1556

Recurrent OM- persisten inflammatory reaction Impaired Eustachian tube function causing poor aeration of middle ear Adenoid infection or hypertrophy

Question 1557

Risk factors for OM with effusion

Answer 1557

Down’s, cleft palate CF Primary ciliary dyskinesia Allergic rhinitis RF for AOM

Question 1558

What happens in children with DS and cleft palate in terms of ears?

Answer 1558

Screened regularly for OME

Question 1559

Gold standard for Dx of OME

Answer 1559

Otoscope * Ear drum dull retracted * Fluid level * Loss of light reflex * May not show evidence of inflammation Confirmed my tympanometry and audiometry (\>4 years)

Question 1560

Mx of OME

Answer 1560

Watch and wait Ask parents to slow speech and face child when talking If symptoms persist refer to ENT, refer all DS and cleft palate to ENT urgently Nonsurgical techniques: Close observation Hearing aids Autoinflation Surgical: Grommets Adenoidectomy

Question 1561

Def: OM chronic supparative

Answer 1561

According to WHO * a chronic inflammation of the middle ear and mastoid cavity, which presents with recurrent ear discharges (otorrhoea) through a tympanic perforation More a condition for the adults…

Question 1562

Cx of chronic supparative OM

Answer 1562

If left untreated, infection in chronic suppurative otitis media may spread extracranially causing * facial paralysis * * or intracranially causing * cerebral abscess

Question 1563

Ear discharge without pain History of Acute OM There may be hearing loss

Answer 1563

Chronic supparative otitis media

Question 1564

Answer 1564

Chronic supparative otitis media with perforation

Question 1565

Mx of chronic supparative OM

Answer 1565

Refer those with signs of infection beyod ear urgently Refer all ? Clean ear and give Abx Advise against getting ear wet e.g. in swimming pools

Question 1566

Def: otitis externa

Answer 1566

Inflammation of the external ear canal Can be localised- a folliculitis Diffused- inflammation of skin and subdermis Can be acute \< 3 weeks Or chornic \> 3 months (Malignant OE- spreads to boone causing bone infections

Question 1567

Aetiology of otitis externa

Answer 1567

Bacterial * pseudomona S. aureus Fungal Sebhorrhoeic dermatitis- ass with dandruff scaling Contact dermatitis Ear trauma Excessive moisture- swimming in polluted water Chemicals- hair dye, hair spray

Question 1568

Pain Itch Discharge Red swelling in ear cancal- pus filled

Answer 1568

Otitis externa

Question 1569

Mx of otitis externa

Answer 1569

Remove aggravating factors Topical acetic acids More severe cases topical Abx with topical steroids

Question 1570

Def: sinusitis

Answer 1570

Infection of paranasal sinuses (frontal sinusitis uncommon in first decade of life as they don’t develop until late childhood) Rhino-sinusitis also used Acute \<12 weeks Chronic\> 12 weeks

Question 1571

Aetiology sinusitis

Answer 1571

Commonly occurs after a viral URTI- secondary bacterial ifnection S. pneumoniae H influenza M. catarrhalis Chronic may result from LT alterations of parasinus structure

Question 1572

Nasal discharge Blockage congestions Pain may develop May have fever Chornic * Pain may not be a feature * Loss of smell Exacerbation to acute sx with background chornic problems

Answer 1572

Rhinits

Question 1573

What differentaites sinusitis from sinusoidal tumour

Answer 1573

Blood stained discharge

Question 1574

Mx of sinusitis

Answer 1574

Acute: self-limiting Decongestants Paracetamol, ibuprofen, irrigating with slaine may provide relief Chronc: Advise to control associated symptoms 3m of intranasl steroid Irrigation Refer if orbital or cranial complications

Question 1575

Def: rhinitis

Answer 1575

an inflammatory disorder of the nose which occurs when the membranes lining the nose become sensitized to allergens

Question 1576

Sneezing Nasal blockage, discharge Itching Bilateral eye swelling may be present

Answer 1576

Rhinitis

Question 1577

Mx rhinitis

Answer 1577

Non-sedating anti-histamines Nasal topical corticosteroids Nasal decognestion

Question 1578

Problems assocaited with hearing difficulty

Answer 1578

LD Neurological disorders Visual deficits

Question 1579

What are the soft tissue injuries that can occur during birth

Answer 1579

Cephalhaematoma Caput succedaneum Chignon

Question 1580

With what is cephaloheamatoma associated?

Answer 1580

Ventouse delivery

Question 1581

A sebperiosteal haemorrhage that is soft on palpation

Answer 1581

Cephalohaematoma

Question 1582

Cephalohaematoma characteristic

Answer 1582

Doesn't cross the suture lines No discolouration

Question 1583

Clinical course of cephalohaematoma

Answer 1583

May increase in size after birth and can take a few weeks to resolve Associated with skull # which may be underneath

Question 1584

Cause of caput succedaneum

Answer 1584

Mechanical injury from skull pushing against a narrowed cervix

Question 1585

Characteristics of caput succedaneum

Answer 1585

Subcutaenous Crosses the midline and sutures of the skull Can present with discolouration and poorly defined edges

Question 1586

Clinical course of caput succedaneum

Answer 1586

Presents at its largest size at birth and takes a few days to resolve

Question 1587

What is the ddx for cephalohaematom

Answer 1587

Cranial meningocele: incomplete causes neural herniation

Question 1588

Characteristics of cranial meningocele

Answer 1588

Pulsates and increased pressure on crying

Question 1589

Diffuse boggy swelling that can extend from orbits to occuput and spreads laterally towards ears

Answer 1589

Subaponeurotic haemorrhage aka sublgeal haemorrhage

Question 1590

Answer 1590

Subaponeurotic haemorrhage

Question 1591

Characteristics of subaponeurotic haemorrhage

Answer 1591

Bleed between periosetum and aponeurosis Develops over hours to days with insiduous growth Traumatic birth history Brusing over hte top

Question 1592

Risk factors for subaponeurotic haemorrahge

Answer 1592

Ventouse Birth trauma Coagulopathy

Question 1593

Cx of subaponeurotic haemorrhage

Answer 1593

Bloods loss may be severe leading to hypovolaemic shock Infection

Question 1594

Mx of subaponeurotic haemorrhage

Answer 1594

Vigilant observation May need transufion if severe blood loss or flud bolus as scalp can hold up to 50% of foetal blood Phototherapy if jaundice develops Ix for coagulopathy

Question 1595

Def: congenital diaphragmatic hernia

Answer 1595

Due to failure of the diaphragm to fuse properly during foetal development leading to the abdominal organs migrating up into the chest cavity

Question 1596

What are the 3 types of diaphragmatic hernia

Answer 1596

Posterolateral Bochdalek's hernia Anterior Morgani's hernia Hiatus hernia

Question 1597

What is the most common type of hiatus hernia

Answer 1597

Bochdalek's hernia

Question 1598

Answer 1598

Question 1599

Aetiology of diaphargamtic hernia

Answer 1599

Genetic Most idiopathic 3% risk of recurrence in future pregnancy

Question 1600

Cx of diapharagmatic hernia

Answer 1600

Pulmoanry hypoplasia

Question 1601

Prenatal features of CDH

Answer 1601

Usually diagnosed prenatally on routine USS Mother presents with polyhdramnios

Question 1602

* Tachypnoae tachycardia * Cyanosis * Asymmetry of chest wall * Displaces apex beat and heart sounds * Infant with respiratory distress * Unable to respond to resuscitation

Answer 1602

?congenital diaphragmatic hernia with pulmonary hypoplasia

Question 1603

Ix in CDH

Answer 1603

CXR

Question 1604

Mx of CDH

Answer 1604

Large NG tube and suction to prevent bowel distension Ventilation Surgical repair once stabilised

Question 1605

Cause of inguinal hernia in children

Answer 1605

Due to patent processus vaginalis through which the bowel herniates

Question 1606

Intermittent swelling in groin or scrotum on straining or crying Irredicuble lump Firm and tender lump Unwell infant with irritability and vomiting

Answer 1606

?Inguinal hernia

Question 1607

Mx of inguinal hernia

Answer 1607

Analgesics and sustained gentle compression If reduciton is impossible then emergency sx to avoid strangulation of bowel

Question 1608

Cx of inguinal hernia

Answer 1608

Recurrence Infarction of testes

Question 1609

Def: hyrodecele

Answer 1609

Abnormal collection of fluid within the remnants of the processus vaginalis

Question 1610

How can hydrocele be classified?

Answer 1610

Simple Communicating

Question 1611

Simple hydrocele

Answer 1611

Accumulation of fluid in tunica vaginalis 1-2% of males affected Usually disppears within 1-2y

Question 1612

Communicating hydrocele

Answer 1612

Persistence of processeus vaginalis allowing peritoneal fluid leakage Normally congenital Can occur in older male infants due to peritoneal dialysis or fluid overload

Question 1613

Cx of hydrocele

Answer 1613

Recurrence Secondary cryptorchidism due to scar formation

Question 1614

Asymptomatic scrotal swelling Bluish dicolouration Non tender Transilluminates May present after viral or GI illness in older boys

Answer 1614

Hydrocele

Question 1615

Ix of hydrocele

Answer 1615

Transillumination

Question 1616

Mx of hydorcele

Answer 1616

Usually resolve If persists beyond 18-24m- sx

Question 1617

What is the most common solid tumour in childhood

Answer 1617

Brain malignancy

Question 1618

What are the different brain tumours that can present in childhood

Answer 1618

Astrocytoma-\> glioblastoma multiforme Medullablastoma Ependyoma Brainstem glioma Craniopharyngioma

Question 1619

* A developmental tumour * Arising from the remenants of Rathke pouch(roof of developing mouth, gives rise to anterior pituitary) * Not truly malignant but grows slowly in the suprasellar region

Answer 1619

Craniopharyngioma

Question 1620

Cx of brain tumour

Answer 1620

Intellectual decline Poor growth Endocrine problems

Question 1621

\<2y/o Bulging fontanelle New onset seizures Persistent vomiting Increase in head size Abnormal eye movement Strabismus

Answer 1621

?Brain tumour

Question 1622

\>2y/o Persistent headache causing EMW Vomiting Mood changes Focal neurology New onset seizures Gait abnormlaity (+spinal mets presenting with back pain, peripheral weakness)

Answer 1622

?Brain tumour

Question 1623

Ix in ?brain tumour

Answer 1623

FBC MRI: child may need sedation Biopsy LP generally not performed

Question 1624

Mx of: Increase in head size Lack of visual following and abnormal eye movements

Answer 1624

Urgent referral

Question 1625

Mx of brain tumour

Answer 1625

Sx: aims to treat- tissue diagnosis Some tumours may not be suitable for sx CTx RTx MRI scans every 6m for first 2y then annually

Question 1626

Risk factors for HL in children

Answer 1626

EBV Previous mononucleosis Hodgkin's HIV Immunosuppression

Question 1627

Risk factors for NHL in childhood

Answer 1627

EBV HTLV-1 HHV8 in HIV Hep C

Question 1628

Painless lymphadenopathy in the neck Lymph nodes firm and large long histroy (over months) Systemic symptoms * Pruritus * Sweating * Weight loss * Fever * Reed-Sternberg cells

Answer 1628

HL

Question 1629

more rapid progression of symptoms * SVC obstruction * Breathlessness * Abdominal distension

Answer 1629

NHL

Question 1630

Gold standard Ix in lymphoma

Answer 1630

Incisional biopsy

Question 1631

Ix in lymphoma

Answer 1631

FBC: to exclude leukaemia or infectious mononculeosis CXR CT to stage

Question 1632

Indicators for urgent referral from primary care in ?lymphoma

Answer 1632

Non-tender and firm LNs larger than 2cm Enlarging Fever and weight loss with axillary and supraclavicular LNs involved URGENT: hepatosplenomgaly, mediastinal or hilar mass on CXR

Question 1633

Mx of lymphoma

Answer 1633

RTx CTx: increases risk of leukaemia ABVD

Question 1634

Def: neuroblastoma

Answer 1634

An embryonal neoplasm arising from neural crest tissue in adrenal medulla dn SNS

Question 1635

Cx of neuroblastoma

Answer 1635

Cord compression HTN Renal insufficiency

Question 1636

Abdominal mass + Bone pain Pallor Fatigue Unexplained fever Generalised lymphadenopathy Skin nodules in children younger than 6 months Unexplained bruising Horner’s syndrome due to thoracic lesions Hypertension- due to pressure on renal artery

Answer 1636

?Neuroblastoma

Question 1637

Ddx neuroblastoma

Answer 1637

Wilm's tumour Lymphoma

Question 1638

Ix in neuroblastoma

Answer 1638

CXR FBC ESR Clotting studies CT MRI to look for spinal involvement

Question 1639

Gold standard dx of neuroblastoma

Answer 1639

Catecholamine byproducts in urine: HVA and VMA BIopsy of lesions

Question 1640

Mx of neuroblastoma

Answer 1640

Localised without mets can be cured surgically Low-risk patients are observed for spontaenous resolution Immediate risk trated with sx, CTx and RTx High risk patients are given multi-agent CTx, RTx, Sx followed by consolidation with high-dose ctx and peripheral blood stem cell rescue

Question 1641

What is the most common childhood malignancy

Answer 1641

Wilm's tumour

Question 1642

Large painless abdominal mass- often incidentally found in otherwise well child Abdominal distension Uncommon sx * abdominal pain * anorexia * anaemia if haemorrhage into mass * haematuria HTN

Answer 1642

Wilm's tumour

Question 1643

What gene is assocaited with WIlm's tumour

Answer 1643

WT1

Question 1644

Ix in Wilm's tumour

Answer 1644

USS and or CT/MRI Intrinsic renal mass distorting normal structure

Question 1645

Mx of WIlm's tumour

Answer 1645

Initial CTx followed by nephrectomy RTx reserved for advanced disease

Question 1646

Def: rhabdomyosarcoma

Answer 1646

Form of soft tissue sarcoma Orginiates from primitive mesenchymal tissue

Question 1647

Cx of rhabdomyosarcoma

Answer 1647

Mets to lung, liver, bone or bone marrow associated with poor prognosis

Question 1648

Head and neck most common site * Proptosis * Nasal obstruction * Blood-stained nasal discharge Genito-urinary * Dysuria * Urinary obstruction * Scrotal mass * Blood stained vaginal discharge Any unexplained lump Non tender Progressively getting bigger

Answer 1648

?Rhabdomyosacroma

Question 1649

Ix in rhabdomyosarcoma

Answer 1649

FBC- anaemia Scans for mets Urinalysis Biopsy: Dx, MyoD1 molecular study

Question 1650

Mx of rhabdomyosarcoma

Answer 1650

Sx often not done as margins ill defined Combination of CTx, Sx and RTx

Question 1651

F/U in bone tumours

Answer 1651

Every 3m for 2 years

Question 1652

Features of retinoblastoma

Answer 1652

Malignant tumour of retinal cells Tumour develops in retinal cells which are dividing rapidly in eraly life Normally occurs before the age of 5 as retina is fully developed by this age Can be multifocal

Question 1653

Bilateral retinoblastoma

Answer 1653

Hereditary

Question 1654

Unilateral retinoblastoma

Answer 1654

20% are hereditary

Question 1655

What gene is implicated in retinoblastoma

Answer 1655

Rb1

Question 1656

New squint Change in visual acuity FHx White pupillary reflex

Answer 1656

Retinoblastoma

Question 1657

Mx of retinoblastoma

Answer 1657

CTx if bilateral to shirnk tumours followed by local laser treatment of retina RTx for advanced disease or recurrence Most are cured although many are visually impaired

Question 1658

Ix in retinoblastoma

Answer 1658

MRI Pupillary light reflex

Question 1659

Langherans cell histiocytosis

Answer 1659

Disorder of dendirtic cells Rare disorder of abnormal proliferation of histiocytes

Question 1660

Bone lesions * at any age * presents with pain, swelling and sometimes fracture Diabetes insipidus * hypothalamic infiltration Can be systemic * Seborrhoeic rash- skin * Involvement of the gums, ears, * Lungs (chest pain, spontaneous pneumothorax) * liver and spleen (heptosplenomegaly) Weight loss

Answer 1660

Langherans cell histiocytosis

Question 1661

Ix in Langherans cell histiocytrosis

Answer 1661

FBC Clotting stuides U&E Biopsy

Question 1662

Multinucleated langherans cells, histiocytes and eosinophils on biopsy

Answer 1662

Langherans cell histiocytosis

Question 1663

Answer 1663

langherans cell histiocytosis Lytic lesion with well-defined border

Question 1664

Advice for preventative measures in UTI

Answer 1664

High fluid intake Regular voiding/double micturition Prevent constipation Good perineal hygiene Lactobacillus acidophilus

Question 1665

How can enuresis be classified

Answer 1665

Daytime Sceondary

Question 1666

Def: daytime enuressi

Answer 1666

Lack of bladder control during the day in a child old enough to be continent

Question 1667

Aetiology of daytime enuresis

Answer 1667

Lack of attention to bladder sensation Detrusor instability Neuropathic bladder UTI Constipation Ectopic uretur: constant dribbling, child is always damp

Question 1668

Aetiology of secondary enuresis

Answer 1668

Most commonly emotional upset UTI Polyuria from osmotic diuresis or a renal concentrating disorder

Question 1669

What are the signs of neuropathic bladder

Answer 1669

Distended bladder Abnormal perineal sensation Abnormal leg reflexes and gait

Question 1670

What is suggestive of an ectopic uretur

Answer 1670

Dry at night but wet on getting up

Question 1671

Ix in daytime enuresis

Answer 1671

Urin MCS USS Urodynamic studies XR spine MRI

Question 1672

Ix in secondary enuresis

Answer 1672

Urine: infection, glycosuria, proteinuria Urine concentrating ability: early morning urine osmolality USS of renal tract

Question 1673

Treatment of daytime enuresis

Answer 1673

Treat underlying cause If no neurological cause: star charts, bladder training, pelvic flood exercises Anticholinergic drugs e.g. oxybutin

Question 1674

Def: nephrotic syndorme

Answer 1674

Heavy proteinuria Hypoalbuminaemia Oedema Often accompanied by hyperlipidiaemia

Question 1675

How can nephrotic snydome in children be classified

Answer 1675

Steroid-sensitive Steroid resistant Congenital nephrotic syndrome

Question 1676

Features of steroid-sensitivity nephrotic syndrome

Answer 1676

85-90% of cases resolve with corticosteroid therapy Common in asian boys, wealy associated with atopy Often precipitated by respiratory infection

Question 1677

Features of congenital nephrotic syndrome

Answer 1677

Rare, presents in first 3m Recessive inheritance Consanguinous High mortality due to complications from hypoalbuminaemia

Question 1678

Cx of nephrotic syndrome

Answer 1678

**Hypovolaemia** – Abdo pain, feels faint. Low urinary sodium and high haematocrit. Requires urgent treatment with **i.v. albumin** **Thrombosis** – urinary losses of antithrombin, steroid therapy, etc **Infection** – from capsulated bacteria, pneumococcus **Hypercholestrolaemia**

Question 1679

**Periorbital oedema** particularly on waking (earliest sign) **Scrotal/vulval, leg and ankle oedema** **MASSIVE proteinuria** **Hypoalbuminaemia** with corresponding hyperlipidaemia **Ascites** **Breathlessness** due to **pleural effusions** and **abdominal distension**

Answer 1679

Nephrotic syndrome

Question 1680

Electron microscopy shows fusion of podocytes

Answer 1680

Minimal change disease Steroid-sensitive nephrotic syndrome

Question 1681

Ix in nephrotic syndrome

Answer 1681

**Urine dipstick** **FBC, ESR, U&Es, Creatinine, Albumin** **Complement levels (C3, C4)** **ASOT** or **anti-DNAse B titres** and **throat swab** **Urine MCS** **Urinary sodium concentration** **Hepatitis B and C screen** **Malaria screen** (if positive travel history)

Question 1682

Mx of steroid-sensitive nephrotic syndrome

Answer 1682

Oral corticosteroids: 60mg/m^2/d prednisolone Reduce to 40 on alternate weeks after 4w. If unresponsive: renal biopsy

Question 1683

What are some steroid-sparing treatments for steroid-sensitive nephrotic syndrome and when might they be used

Answer 1683

If steroid-dependant/frequent relapses Cyclophosphamide Tacrolims/ciclosporin Levamisole Mycophenolate mofetil

Question 1684

Treatment of steroid-resistant nephrotic syndrome

Answer 1684

Refer to paediatric nephrologist Diuretics ACEI Salt restriction NSAIDs

Question 1685

Treatment of congenital nephrotic syndrome

Answer 1685

If albuminuria is very severe: unilateral nephrectomy Dialysis until the child is large enough for renal transplant

Question 1686

Causes of nephritic syndrome

Answer 1686

* Post infectious (including streptococcus) * Vasculitis (Henoch-Schönlein purpura or, rarely, SLE, Wegener’s granulomatosis, polyarteritis nodosa, microscopic polyarteritis) * IgA nephropathy and mesangiocapillary glomerulonephritis * Anti-glomerular basement membrane disease (Goodpasture’s Disease) – very rare * Post infectious (including streptococcus) * Vasculitis (Henoch-Schönlein purpura or, rarely, SLE, Wegener’s granulomatosis, polyarteritis nodosa, microscopic polyarteritis) * IgA nephropathy and mesangiocapillary glomerulonephritis * Anti-glomerular basement membrane disease (Goodpasture’s Disease) – very rare

Question 1687

_Clinical features_ * Reduced urine output and volume overload * Oedema (normally periorbital) * Hypertension which may cause seizures * **Haematuria** and red cell casts , proteinuria

Answer 1687

Nephritic syndrome

Question 1688

Ix in nephritic syndrome

Answer 1688

* Urine microscopy (with phase contrast) and * culture * Protein and calcium excretion * Kidney and urinary tract ultrasound * Plasma urea, electrolytes, creatinine, calcium, * phosphate, albumin * Full blood count, platelets, clotting screen, * sickle cell screen.

Question 1689

If ?glomerular haematuria

Answer 1689

* ESR, complement levels and anti-DNA * antibodies * Throat swab and antistreptolysin O/anti-DNAse B titres * Hepatitis B and C screen * Renal biopsy if indicated * Test mother’s urine for blood (if Alport syndrome * suspected) * Hearing test (if Alport syndrome suspected)

Question 1690

Mx of nephritic syndrome

Answer 1690

Fluid and electrolye balance Diurteics Monitor for rapid deterioriation in renal function

Question 1691

Follows a streptococcal sore throat or skin infection Diagnosed by **evidence of recent infection** (culture of organism, raised ASO/anti-DNAse B titres) and **low complement C3** that return to normal after 3-4 weeks

Answer 1691

Post-streptococcal and postinfectious nephritis

Question 1692

* Characteristic skin rash (buttocks, extensor surfaces of arms and legs, ankles). Initially urticarial becomes maculopapular. Spares trunk. **Cornerstone of diagnosis.** * Arthalgia * Periarticular oedema * Colicky abdominal pain * Glomerulonephritis Occurs in **boys** between the ages of **3-10 years**. Peaks during **winter** and often preceded by **URTI**. Proposed that IgA and IgG complex and deposit in affected organs, activating complement

Answer 1692

HSP

Question 1693

Episodes of **macroscopic haematuria** in association with **upper respiratory tract infections**. Histologically similar to Henoch-Schönlein.

Answer 1693

IgA nephropathy

Question 1694

X-linked recessive disorder that progresses to **end-stage renal failure** by early adult life in males and is associated with **nerve deafness** and **ocular defects**. The mother may have haematuria.

Answer 1694

Alport syndrome

Question 1695

Characteristic symptoms are fever, malaise, weight loss, skin rash and arthropathy with prominent involvement of the respiratory tract

Answer 1695

Wegner's

Question 1696

Characteristic symptoms are fever, malaise, weight loss, skin rash and arthropathy with Renal arteriography demonstrating aneurysms

Answer 1696

PAN

Question 1697

Treatment of vasculitides

Answer 1697

Steroids PLEX IV cyclophosphamide

Question 1698

Predisposing causes of renal stones in childhood

Answer 1698

**à UTI** **à Structural abnormalities of the urinary tract** **à Metabolic abnormalities** most commonly idiopathic hypercalciuria

Question 1699

What most commonly caues phosphate stones

Answer 1699

Proteus

Question 1700

Nephrocalcinosis occurs with

Answer 1700

With hypercalcuria Hyperoxaluria Distal renal tubular acidosis

Question 1701

DMSA

Answer 1701

Static scan of renal cortex Detects functional defects but very sensitive so need to wait 2m after UTI to avoid dx false scars

Question 1702

Draw protocol for antenatally diagnosed UT anomalies

Answer 1702

Question 1703

Nitrate

Answer 1703

Positive result likely to indicated a UTI NB some children with a UTI are nitrate negative

Question 1704

LE

Answer 1704

May be present in children with UTI but may also be negative Present in children with febrile illness without UTI Positive in balantitis and vulvovaginitis

Question 1705

LE + N +ve

Answer 1705

Regard as UTI

Question 1706

LE -ve Nitrate +ve

Answer 1706

Start abx treatment Dx depends on urine culture

Question 1707

LE +ve Nitrate -ve

Answer 1707

Only start abx if clinical evidence of UTI Dx depends on culture

Question 1708

LE + N -ve

Answer 1708

UTI unlikely Repeat or send urine for culture

Question 1709

Causes of proteinuria

Answer 1709

Orthostatic proteinuria Glomerular abnormalities: Minimal change GN Abnromal glomerular BM Increased GFR pressure Reduced renal mass HTN Tubular proteinuria

Question 1710

What are the causes of steroid-resistant nehprotic syndorme

Answer 1710

Focal segmental glomerulosclerosis Mesngiocapillary GN Membarnous nephropathy

Question 1711

Most common cause of steroid-resistnat nephrotic syndrome

Answer 1711

Focal segmental glomerulosclerosis

Question 1712

Px of focal segmental glomerulosclerosis

Answer 1712

30% progress to ESRF 20% respond to steroid sparing agents Recurrence post-transplant common

Question 1713

Nephrotic syndrome: More common in older children Haematuria and low complement level present

Answer 1713

Mesnagiocapillary glomerulonephritis (membranoproliferaive)

Question 1714

Nephrotic syndrome: Associated with Hep B May preced SLE

Answer 1714

Membranous nephropathy

Question 1715

How can the causes of haematuria be classified?

Answer 1715

Non-glomerular Glomerular

Question 1716

Draw the causes of haematuria

Answer 1716

Question 1717

How can the causes of HTN be classified?

Answer 1717

Rneal Coarctation Catecholamine excess Endocrine Essential HTN

Question 1718

Draw the causes of HTN

Answer 1718

Question 1719

How can the causes of palpable kidneys be classified

Answer 1719

Unilateral Bilateral

Question 1720

Draw the causes of palpable kidneys

Answer 1720

Question 1721

Draw the causes of acute renal failure

Answer 1721

Question 1722

What are the metabolic abnormlities seen in acute renal failure

Answer 1722

Metabolic acidosis Hyperphosphataemia Hyperkalaemia

Question 1723

How to correct metabolic acidosis in acute renal failure

Answer 1723

Sodium bicarb

Question 1724

How to correct hyperphosphattaemia in acute renal failure

Answer 1724

Ca carbonate Dietary restriction

Question 1725

How to correct hyperkalaemia in acute renal failure

Answer 1725

If ECG changes: calcium gluconate Salbutamol (nebulised or IV) Calcium exchange resin Glucose and insulin Dietary restriction DIalysis

Question 1726

What is the most common cause of chronic renal failure

Answer 1726

Structural malformations

Question 1727

What are the common causes of chronic renal failure

Answer 1727

Structural malformation GN Hereditary nephropathies Systemic disease Miscellaneous/unknown

Question 1728

Draw the causes of diurnal enuresis

Answer 1728

Question 1729

What are the causes of frequent and excessive urination

Answer 1729

UTI Psychogenic DM DI Chronic renal failure

Question 1730

Causes of haematuria

Answer 1730

UTI Trauma Acute GN Stones and hypercacliruia Congenital anomalies Tumour Coagulopathy Exercise Drugs

Question 1731

Answer 1731

Question 1732

Answer 1732

Question 1733

Mx of nephrotic syndrome

Answer 1733

Hospitalise, monitor weight and urinary protein loss Moderate fluid and salt intake Steroids to induce remission Low dose steroids for 3-6m Prophylactic penicllin Cyclophosphamide if steroids ineffective

Question 1734

Barrter syndrome

Answer 1734

Bartter syndrome is a rare inherited defect in the thick ascending limb of the loop of Henle. It is characterized by low potassium levels (hypokalemia),[1] increased blood pH (alkalosis), and normal to low blood pressure. There are two types of Bartter syndrome: neonatal and classic. A closely associated disorder, Gitelman syndrome, is milder than both subtypes of Bartter syndrome.

Question 1735

underdeveloped lungs, which can cause severe breathing difficulties soon after birth high blood pressure (hypertension) excessive peeing and thirst problems with blood flow through the liver, which can lead to serious internal bleeding a progressive loss of kidney function, known as chronic kidney disease (CKD)

Answer 1735

ARPKD

Question 1736

Why is ADPKD called adult PKD?

Answer 1736

Although children are born with the condition, ADPKD does not usually cause any noticeable problems until the cysts grow large enough to affect the kidneys' functions. In most cases, this doesn't occur until 30-60 years of age.

Question 1737

Def: overweight

Answer 1737

91st BMI centile

Question 1738

Def: obesity

Answer 1738

98th BMI centile

Question 1739

When to start to use adult parameters for BMI assessment

Answer 1739

\>12y/o

Question 1740

What are the BMI centiles for population monitoring

Answer 1740

Overweight: 85th BMI centile 95th BMI centile= obese

Question 1741

Def: Pickwickian syndrome

Answer 1741

besity hypoventilation syndrome (also known as Pickwickian syndrome) is a condition in which severely overweight people fail to breathe rapidly enough or deeply enough, resulting in low blood oxygen levels and high blood carbon dioxide (CO2) levels. Many people with this condition also frequently stop breathing altogether for short periods of time during sleep (obstructive sleep apnea), resulting in many partial awakenings during the night, which leads to continual sleepiness during the day.[1] The disease puts strain on the heart, which eventually may lead to the symptoms of heart failure, such as leg swelling and various other related symptoms. The most effective treatment is weight loss, but it is often possible to relieve the symptoms by nocturnal ventilation with positive airway pressure (CPAP) or related methods.

Question 1742

Mx of obese child

Answer 1742

Exclude organic pathology Health eating and encourage regular meals, discourage snacking Increase physical activity Management doesn't involve weight loss, rather weight maintenance so children "grow" into their weight Further treatment is reserved for those \>40BMI Drug treatment is with orlistat or metformin (especially if insulin resistance is apparent)

Question 1743

Ix in obese child

Answer 1743

BP Blood glucose, insulin levels Cholesterol TGs LFTs BMI TFT and other endocrine function tests

Question 1744

Cx of obesity

Answer 1744

Endocrine – T2DM, metabolic syndrome, insulin resistance CVS – hypertension Resp – obesity hypoventilation syndrome (aka Pickwickian syndrome), obstructive sleep apnoea, snoring, daytime somnolence, asthma exacerbation GI – gall bladder disease Neuro – idiopathic intracranial hypertension Gynaecological – early menarche, Polycystic ovarian syndrome Orthopaedic – slipped upper femoral epiphysis, bow legs (varus), foot problems Malignancies – Colon CA, breast CA Psychological – self-esteem, depression, more likely to experience downward social mobility

Question 1745

What is the most common cause of obesity

Answer 1745

Nutritional

Question 1746

What are the rare causes of obesity in childhood

Answer 1746

Hypothyroidism Cushing's sydnrome Various genetic syndromes

Question 1747

Def: delayed pubertify

Answer 1747

Absence of pubertal development by age 14 in girls and 15 in boys

Question 1748

What are the causes of delayed puberty

Answer 1748

Congenital delay of growth and puberty Hypogonadotrophic hypognoadism (low gonadotrophin) Hypergonadotrophic hypogonadism (high GTH)

Question 1749

Answer 1749

Question 1750

Def: constitutional delay of growth and puberty

Answer 1750

Variation of normal timing of puberty Delayed puberty that is familial often having occured in parent of the same gender

Question 1751

Ix in CDGP

Answer 1751

**Good Hx** including FHx – look for patterns of female menarche ages, evidence of gonadal dysplasia syndromes in the child. Check on social/educational aspects - ?neglect. **O/E:** height, weight & plot. Pubertal staging inc testicular volume . Look for dysmorphic features, general examination including fundoscopy and visual fields, look for signs of chronic disease. Calculate mid-parental heightPlotting present and previous weights/heights on growth charts **Bloods for chronic disease**: FBC, ferritin, renal function, U/Es, Coeliac screening (serum TTG), urine dip for blood/proteins **Bloods for disorders of gonadal axis:** karyotyping, basal FSH/LH/E2/T4, pelvic USS for girls, bone age, GnRH test, PRL, GH, MRI/CT for pituitary if indicated

Question 1752

Mx of CDGP

Answer 1752

Medication not normally needed Androgens and oestrogens can be used to induce puberty

Question 1753

Def: congenital hypothyroidism

Answer 1753

Lack of thyroid hormones present from birth – if not detected and treated early can lead to irreversible neurological damage and poor growth. \*Only small amount of thyroxine transfer from M to F (but severe maternal hypothyroid can cause brain damage), fetal thyroid produces inactive T3. After birth surge in TSH à rise in T4 and T3 à fall to adult levels within a week

Question 1754

Causes of congenital hypothyroidism

Answer 1754

* Maldescent or athyrosis, thyroid aplasia, hypoplasia, ectopic thyroid tissue * Dyshormonegenosis: inborn error of synthesis, TSH unresponsiveness, defects in thyroglobulin * Hypothalamic or pituitary dysfunction: usually panhypopituitary (hypoglycaemia, undescended testes, micropenis noticeable first); hypothalamic tumours/ischaemic mg/congential defects Iodine deficiency

Question 1755

**Symptoms:** failure to thrive, feeding problems, somnolence, lethargy, constipation, delayed development **Signs:** Pale/cold/mottled skin, coarse facies, large fontanelle, large tongue, hoarse cry, goitre, umbilical hernia, myxoedema, oedema of genitalia and extremities, nasal obstruction, prolonged jaundice, hypotonia, cardiomegaly, bradycardia, pericardial effusion, failure of fusion of distal femoral epiphyses, refractory anemia

Answer 1755

Congenital hypothyroidsim

Question 1756

Mx of congential hypothyroidism

Answer 1756

Start thyroxine at 203weeks of age, OD L-thyroxine titrated to TFTs, growth. Repplacement is lifelong, minotr: TFTs, cross-sectional reference growth charts, developmental milestones, mental development

Question 1757

* Rapidly enlarging thyroid gland – SOB, dysphagia, goitre will remain unchanged for decades * Occasionally mild thyrotoxicosis at the start of the disease * Short stature, growth failure, delayed puberty * Cold intolerance, cold peripheries * Dry skin; thin dry hair; pale puffy eyes with loss of eyebrows * Slow-relaxing reflexes * Constipation * Bradycardia * Obesity * Associations: slipped upper femoral epiphysis, deterioration in schoolwork, leranign difficulties; other autoimmune conditions (alopecia areata, hypogonadism etc) If extreme, can lead to myxoedema coma, encephalopathy, hyperlipidaemia

Answer 1757

Hashimoto's autoimmune thyroiditis

Question 1758

Ix in CNS tumours

Answer 1758

* Endocrine studies * \*Macroprolactinaemia (prolactin of high molecular mass, mostly complexes of monomeric prolactin with immunoglobulins) - suspect this if there is a high prolactin level with no symptoms (eg normal menstrual cycles). The serum sample should be treated with polyethylene glycerol (PEG) to precipitate out the macroprolactin * Imaging- plain Xray will show calcified cyst in/above pituitary fossa (common in children); CT, MRI * Pituitary stimulation test to assess need for pituitary hormone replcament * Visual fields test Consider psychiatric assessment if appropriate

Question 1759

Treatment of CNS tumours

Answer 1759

* Surgical resection- trans-sphenoidal for lesions within sella turcica and ACTH-secreting adenomas * Hormone-secreting pituitary tumours can be treated with meds: bromocriptine/cabergoline for prolactin-secreting adenomas, somatostatin analogues for Gh secreting adenomas * Intracystic chemotherapy or radiotherapy (brachotherapy) if sx not possible Panhypopituitarism treated with appropriate replacements

Question 1760

Def:CAH

Answer 1760

Disorder of cortisol biosynthesis – deficiency of an enzyme in the steroid biosynthesis pathways (\>90% are of 21-hydroxylase) that causes more precursors to be converted to adrenal androgens. Characterised by cortisol deficiency, with or without aldosterone deficiency and androgen excess. (because autosomal recessive, Mendelian pattern of inheritance and therefore spectrum of phenotypes)

Question 1761

Aetiology of CAH

Answer 1761

21-hydroxylase gene is on 6p21 within HLA histocompatibility complex In foetus, cortisol deficiency à ACTH production à overproduction of adrenal androgens. Deficiency of mineralocorticoids causes salt-losing crisis

Question 1762

What are the features of the calssic form of CAH

Answer 1762

severe form, subclassified as salt-losing or non-salt losing(simpelr virilising)

Question 1763

* Baby girls: virilisation or external genitalia, clitoris hypertrophy, variable fusion of labia, common urogenital sinus in palce of a separate urtethra and vagina. Prader staging 1-5 to classify severity of virilisation. But uterus, ovaries normal and intact. * Baby boys: enlarged penis, scrotum pigmented. Non-salt losing from – early virilisation * Boys with salt-losing form: present at day 7-14 with vomiting, floppy, weight loss, circulatory loss, hyponatremia, hyperkalemia, shock, acidosis Both, non-salt losing: increase muscle build, precocious puberty

Answer 1763

CAH

Question 1764

Early puberty, young women – infertility, hirsutism, oligo/amenorrhoea, PCOS, acne, psychosexual issues from xs testosterone, increased muscle build; males early puberty

Answer 1764

Nonclassic CAH

Question 1765

Low Na, high K, metabolic acidosis, hypoglycaemia

Answer 1765

Salt-losing CAH

Question 1766

Dx of CAH

Answer 1766

High levels levels of 17α-hydroxyprogesterone (precursor) in blood – 21 hydroxylase deficiency, send after 48h because high in all babies at birth Corticotrophin stimulation test used to assess borderline cases and the gold std for Dx in onoclassic form

Question 1767

Treatment of adrenal crisis

Answer 1767

Saline Dextrose IV hydrocortisone

Question 1768

Treatment of classic CAH

Answer 1768

* glucocorticoids lifelong to suppress ACTH. Fludrocortisone to replace mineralocrticoids. Infants will need NaCl supplementation before weaning. When under phjsyical stress (febrile illness, trauma, injury etc), will need increased dose of hydrocortisone, IV hydration, glucose monitoring (MEDICALERT BRACELET). Regular monitoring of growth, skeletal maturity, plasma androgens, 17s-hydroxyprogestoerone to titrate between xs ACTH and comrpomisd growth.

Question 1769

Treatment of CAH: females with virilisation

Answer 1769

sometimes need corrective surgery before age 1, surgery to reduce clitoris and vaginoplasty before intercourse. Often experience psychosexual problems

Question 1770

Mx of mothers at risk of carrying a child with CAH

Answer 1770

maternal dexamethasone after prenatal diagnosis to reduce virilisation, balance with risk of IUGR

Question 1771

Answer 1771

Question 1772

WHat is significant about precocious puberty in boys

Answer 1772

More likely to be pathological rather than physiological

Question 1773

Draw the causes of precocious puberty

Answer 1773

Question 1774

Draw the causes of delayed puberty

Answer 1774

Question 1775

Def: premature puberty

Answer 1775

Development of secondary sexual characteristics before age 8 in girls and 9 in boys When accompanied by growth spurt= prcocious puberty

Question 1776

Draw the classification of renal tract abnormalities

Answer 1776

Question 1777

How can precocious puberty be classified?

Answer 1777

Gonadotrophin dependant Gonadotrophin independant

Question 1778

Precocious puberty in females

Answer 1778

Females- normally idiopathic or familial. Organic causes rare and associate with dissonance (change in sequence) – suggesting XS androgens, rapid onset, nueorlogical symptoms and signs (eg neurofibromatosis)

Question 1779

Precocious puberty in males

Answer 1779

normally an organic cause, intracranial tumours especially

Question 1780

Gonadotrophin dependent precocious puberty

Answer 1780

Idiopathic/familial CNS abnormalities Hypothyroid

Question 1781

Gonadotrophin independant precocious puberty

Answer 1781

Adrenal: tumour CAH Ovarian tumour: granulosa cell Testicular tumour: leydig Exogenous steroids

Question 1782

Ix in precocious puberty

Answer 1782

Full hx including family hx Examine testes in males Tanner staging of puberty Levels of sex steroid: pubertal levels found in gonadotrophin independent puberty LH and FSH to determine aetiology TFT Adrenal precursors if ?CAH hCG if ?hCG secreting tumours Urinary 17-ketosteroids- adrenal andorgens Cranial MRI ?intracranial tumour

Question 1783

Bilateral testes enalrgement in precocious puberty

Answer 1783

Gonadotrophin release: intracranial lesion

Question 1784

Small testes in precocious puberty

Answer 1784

Adrenal andorgen production

Question 1785

Unilateral testicle enlargement in precocious puberty

Answer 1785

Tumour

Question 1786

Gold std dx of precocious puberty

Answer 1786

Pelvic USS Bone XR bone aging Leuprolide acetate stimulation testing: accurately predict pubertal progression

Question 1787

If bone age is within 1 year of chronological age

Answer 1787

Puberty has not started/only just started If \>2y advanced, puberty has been present for one year/is progressing rapidly

Question 1788

Mx of gonadotrophin dependant precocious puberty

Answer 1788

GnRH analogues

Question 1789

Mx of gonadotrophin-independent precocious puberty

Answer 1789

inhibitors or androgen release(ketoconazole)/action (cyproteone acetate); oestrogen action(tamoxifen in mcune-albright, medroxyprogesterone) or production

Question 1790

Mc-Cune Albright Syndrome

Answer 1790

consists of at least two features of the triad of:[1] Polyostotic fibrous dysplasia. Café-au-lait skin pigmentation. Autonomous endocrine hyperfunction (including precocious puberty, thyrotoxicosis, pituitary gigantism and Cushing's syndrome).

Question 1791

Def: premature pubarche

Answer 1791

Pubic hair develops \<8 in girls,\<9 in boys but with no other signs of sexual development

Question 1792

Aetiology of premature pubarche

Answer 1792

Premature adrenarche – adrenal androgens (along with other symptoms) Exogenous androgens – contact with topical preparations PCOS in girls?

Question 1793

Ix in premature pubarche

Answer 1793

Pelvic USS Bone age Urianry 17-ketosteroids to exclude adrenal andorgens/tumour ?late onset CAH

Question 1794

Def: short stature

Answer 1794

Height below the 2nd centiel i.e. 2SD below the mean

Question 1795

Ix in short stature

Answer 1795

**Hx:** include prenatal/perinatal Hx, maternal health and habits during pregnancy, check Red Book. Gnereal nutrition, feeding problems, special diets etc. Chronic disease and medication ( WATCH OUT FOR PHYSICAL SYMPTOMS – eg Coeliacs can present with faltering growth without any GI symptoms). Look for signs of developmental delay, indications of child abuse. **O/E:** Measure height with calibrated stadiometer, take weight – chart. Thorough physical examination to look for dysmorphic features too . Include fudoscopy – check for other signs of hypopituitarism – papilloedema, visual field defects. Calculate mid parental/expected heigh **Initial bloods:** FBC (anaemia of chornic disease, Coeliac), U/Es (renal disease, electrolyte imbalance), LFT, TFT (hypothyroid), Urinalysis, ESR, CRP (Crohn), ca/phosphate/alk phos (renal and bone disorders). Speicific test – coeliac (anti-TTG, endomysial IgA), 0900h cortisonl and dexamethasone suppression test (cushing’s), CF, GH provocation tests with insulin/glucagon (GH deficiency), hypothyroid, vit D deficiency, IGF-1, CT/MRI (craniopharyngioma/intracranial tumour) **Bone age:** can help predict final adult height by estimating skeletal maturation

Question 1796

When to refer in short stature

Answer 1796

**Refer if:** height fails to progress along appropriate centile curve, decreased growth velocity, dysmorphic/syndromic features, bone age delayed by \> 2SD Deal with underlying cause of short stature **NICE recommends somatotrophin (GH analogue)** for: Gh deficiency, Turner’s, Prader-Willi, chronic kidney disae, small for gestational age + subsequent growth failure at \>4yo, short stature homeobox gene (SHOX) deficiency

Question 1797

Draw the classification of causes of short stature

Answer 1797

Question 1798

Answer 1798

Question 1799

Answer 1799

Question 1800

Draw the causes of anaemia in infants and children

Answer 1800

Question 1801

Draw the simple diagnostic approach to anaemia in children

Answer 1801

Question 1802

What is Diamond-Blackfan syndrome?

Answer 1802

Congenital red cell aplasia

Question 1803

Features of IDA in childhood

Answer 1803

Common infants and toddlers, especially if of Indian descent Usually dietary in origin Due to high Fe demands for growth and body stores. Will occur if infants are weaned at 6m of age Treated with dietary advice and oral Fe therapy for at least 3m

Question 1804

Features of Beta-thal major

Answer 1804

Mutation of beta-globin results in an inability to produce HbA Condition is fatal without regular blood transfusions although these can cause Fe overload Treat with desferrioxamine or oral iron chelation

Question 1805

What are some dietary soruces of iron?

Answer 1805

High Fe: Red meat Liver, kidney Oily fish Average Fe: Pulses, beans, peas Fortitifed breakfast cereals Wholemeal products Dark greens Dried fruit Nuts and seeds

Question 1806

What food should be avoided in toddlers (in the context of IDA)?

Answer 1806

Cow's milk Tea: tannin inhibits Fe uptake High fibres foods: phytases inhibit Fe absorption

Question 1807

What are the drugs and chemicals that can cause haemolysis in G6PD?

Answer 1807

Anitmalarials: Primaquine Quinine Chloroquine Antibiotics: Sulphonamides (co-trimoxazole) Quinolones: ciprofloxacin Nitrofurantoin Aspirin in igh doses Napthalene (mothballs)

Question 1808

Pallor Jaundice Bossing of skull Maxilalry overgrowth Splenomegaly and hepatomegaly

Answer 1808

Beta thalassaemia major

Question 1809

Answer 1809

Facies of beta-thal major

Question 1810

What are the complications of LT blood transfusion in children?

Answer 1810

Fe deposition (most important- all patients) Ab formation (10% nof children) Infection (now uncommon) VEnous access (common problem)

Question 1811

Anaemia Infection Painful crises Acute anaemia Pripaism Splenomegaly

Answer 1811

Sickle cell

Question 1812

Vulnerability to infection in SCCD

Answer 1812

All have marked increase in susceptibility to infection by encapsulated organisms: HiB, pneumococcus Children are also at risk of osteomyelitis caused by Salmonella Consequence of hyposplenism secondary to chronic sickling and splenic microinfarctions

Question 1813

Hand-foot syndrome Dactylitis with swelling and pain of the fingres and or feet from vaso-occlusion

Answer 1813

Painful sickle crises

Question 1814

Manifestations of acut anaemia in SCD?

Answer 1814

Haemolytic crises (?infection) Aplastic crises (parvovirus, temporary) Sequestration crises

Question 1815

LT problems in SCD

Answer 1815

Short stature and delayed puberty Stroke and cognitive problems (subtle neurological damage may occur) Adenotonsillar hypertrophy Cardic enlargement Heart failure Renal dysfunction Pigment gallstones Leg ulcers Psychosocial problems

Question 1816

Anaemia in neonates=

Answer 1816

\<14

Question 1817

Anaemia in 1-12m=

Answer 1817

\<10

Question 1818

Anaemia in 1-12y

Answer 1818

\<11

Question 1819

What are the causes of IDA in childhood?

Answer 1819

Main causes of iron def anaemia are: * Inadequate intake * Malabsorption * Blood loss – three most common causes:- * cow’s milk enteropathy * menstruation * hook worm infection Additional iron is required in infants as they are growing and need to build up iron stores Iron can come from: * Breast milk (low content but 50% of the iron is absorbed * Infant formula- supplemented with iron * Cow’s milk- higher content than breast milk but only 10% absorbed Solids introduced at weaning but poor absorption

Question 1820

Complications of IDA in chidlhood?

Answer 1820

* poorer cognitive, motor and socio-emotional function, * In older pre-school children, development affected causing poorer motor, cognitive and language development and poorer learning performance and behaviour. * Severe iron deficiency associated with thrombotic stroke

Question 1821

1st phase: abdo pain with D&V Apparent recovery 8-16h Systemic involvement: hypotension, mitochondrial poisoning-\> drowsiness

Answer 1821

Fe poisoning

Question 1822

When does IDA become symptomatic?

Answer 1822

\<6-7g/dl

Question 1823

* Tire easily * Infants feed more slowly than usual * Pallor of conjunctivae, tongue or plamar creases * Children may have pica- inappropriate eating of non-food material eg soil, foam rubber

Answer 1823

Symptomatic IDA

Question 1824

Low MCV, MCH Low ferritin

Answer 1824

IDA

Question 1825

Mx of IDA

Answer 1825

Dietry advice Oral iron supplement- continues until Hb is normal and then for a further 3 months to build up iron stores * Sytron- sodium iron edetate * Niferex- pollysaccharride iron complex Failure to respons: * Think compliance * Consider another cause * Malabsorption- coeliac * Chronic blood loss- Meckles diverticulum Can have iron deficiency with normal Hb * Low serum ferritin * Controversial whether to give oral iron * Treatment favoured as iron deficiency affects intellect and behaviour Management tends to be- dietry advice with option of oral iron if parents want

Question 1826

Mx of beta-thal major

Answer 1826

Treatment * Monthly transfusions to avoid effects of ineffective erythropoiesis * Aim to keep Hb\> 10 g/dl * BMT * Should be done in first 2 years of life * Generally successful in those with a HLA- identical sibling (90-95% success) (5% chance of transplant related mortality) * Gene therapy * Remains experimental

Question 1827

Mx of Fe overload

Answer 1827

Iron overload Chelation started when 10-12 transfusions received or ferritin is \>1000micrograms/L Desferrioxamine- parenteral- usually subcut- 5-6 nights a week Poor compliance Effects- Sensorineural deafness Visual distrubances Auditroyr and ophthalmic assessment before starting therapy Vertebral dysplasia Growth impariement- growth monitored Deferiprone Oral chelator Removes cariadc iron more effectively Causes agranulocytosis 1%- full blood count monitoring Transient arthropathy

Question 1828

Draw the pathophysiology of beta-thal major

Answer 1828

Question 1829

What factors exacerbate sickling?

Answer 1829

Reduced O2 tension i.e. hypoxaemia Cold Dehydration Illness Psychological stress

Question 1830

**SCA- homozygous for HbS- HbSS- most severe**

Answer 1830

* Sickle mutation in both B-globin chains * No HbA * Small amounts of HbF

Question 1831

**HbSC disease**

Answer 1831

* HbC- due to a different mutation in B-globin chain * Children inherit one HbS and one HbC * No HbA as no normal B-globins

Question 1832

**Sickle B-thalassaemia**

Answer 1832

* HbS from one parents and B-thal trait from another * No HbA * Symptoms like SCA

Question 1833

**Sickle cell trait**

Answer 1833

* HbS from one parent and one normal B-globin gene * 40% HbS * Have HbA Don’t have sickle cell disease0 carriers

Question 1834

What are the acute cxs that arise a consequence of cell sickling?

Answer 1834

* Acute painful crises * Acute anaemia * Acute chest syndrome * Due to infection- mycoplasma, gram neg and gram pos * Infarction- * Acute osteomyletis – commonly due to salmonella and staph. * Acute renal failure- due to vaso-occlusion, dehydration * Acute stroke- due to infarction- common in children * Acute eye problems- sudden vision loss or change in vision Long term problems * Chornic anaemia due to shorter lifespan of RBC * Gallstones- due to chronic haemolysis * Small infarcts can cause cognitive problems (17%)

Question 1835

ridual tubular spiral bodies’

Answer 1835

?Sickle cell

Question 1836

Primary care mx of scikle cell disease

Answer 1836

**Prophylaxis** * Increased risk of encapsulated organism due to damaged spleen * Fully immunised * Daily oral penicillin in childhood **Once daily folic acid due to increased RBC turnover** **Avoid cold, exercise, dehydration to reduce sickling** **Sickle cell Crisis** If fever and origin known can be managed within community with relevant treatment If only mild crisis -advise parents to : * increase fluid intake * distraction teqhnicues * can prescribe paracetamol and ibuprofen (careful in those with renal impairment) * Codeine phosphate if those not effective

Question 1837

Secondary management of SCA

Answer 1837

**Sickle cell crises** Admit if suspect sickle- cell crisis with high fever Child will have dactylitis as a presentation Admit if acute chest syndrome- dyspnoea, confusion, hypoxia * IV fluids * Strong opiates * Spirometry every 2 hours for those with ACS **Transfusions** * the normal steady level of Hb for the patient should be known as will be lower than normal * transfusions given if decreased below this level * if Hb increases from their steady state level can cause hyperviscosity * transfusions normally given if- splenic sequestration or decreased RBC (red cell aplasia) **Exchange transfusions** * to reduce percentage of HbS and reduce sickle related complications given in sepsis, acute stroke, ACS

Question 1838

Neonatal Jaundice- 1st three days of life * most common cause of neonatal jaundice and requires exchange transfusion Acute intravascular haemolysis * fever * malaise * dark urine * Hb levels fall rapidly (\<5 g/dl over 24-48 hrs) precipitated by * infection (most common) * drugs * fava beans * naphthalene in moth balls Gallstone history Normal between episodes

Answer 1838

G6PD- X linked recessive

Question 1839

FBC Raised reticulocytes Blood film- Heinz bodies Direct antiglobulin test negative

Answer 1839

G6PD

Question 1840

What is important about measuring G6PD in RBCs?

Answer 1840

Measuring G6PD in RBC * during a haemolytic episode G6PD may be raised as increased reticulocytes produced by BM to compensate- retics have higher G6PD one measurement needed between episodes

Question 1841

What accounts for 80% of leukaemias in children?

Answer 1841

ALL

Question 1842

Def: ALL

Answer 1842

Accounts for 80% of leukaemias in children Clonal prliferations of cells from the lymphoid progenitor cells Can be T-cell origins but most are B-cell Lymphoid precursors proliferate and replace normal cells Blasts (immature cells) seen in the peripheral circulation Peak age is about 2-4 years

Question 1843

* Fatigue, dizziness and palpitations * Severe and unusual bone and joint pain * Recurrent and severe infections (oral, throat, skin, perianal infections commonly) * Fever without obvious infection * Left upper quadrant fullness and early satiety due to splenomegaly (10-20%) * Dyspnoea (due to anaemia) * Headache, irritability * Thrombocytopenia- bruising, nose bleeds

Answer 1843

?ALL

Question 1844

What is the gold standard for Dx of ALL?

Answer 1844

BM aspirate showing \>20% blasts

Question 1845

Mx of ALL

Answer 1845

**Remission induction** Combination chemo (vincristine, dexamethasone cylclopho) goal is to * To eliminate more than 99% of the initial burden of leukaemic cells * To restore rapidly normal haematopoiesis * To restore previous performance status **Consolidation and CNS protection** Intensive chemo given to consolidate remission * Chemo cannot reach CNS * Intrathecal chemo is given to prevent CNS relapse * IT vincristine, methotrexate **Maintenance** Chemo of modest intensity Continues for at least 3 years after diagnosis * Monthly vincristineand dexameth * Weekly oral methotrexate * Prophylactic co-trimoxazole to prevent PCP * Itrathecal methotrexate After relapse High dose chemo BMT considerations with total body irradiation

Question 1846

Mucous membrane bleeding and skin haemorrhage

Answer 1846

Platelet disorders: vWD

Question 1847

Bleeding into muscles or into joints

Answer 1847

Haemophilia

Question 1848

Scarring and delayed haemorrhage suggestive of

Answer 1848

CT disorders e.g. Marfan's, factor XIII defieicny

Question 1849

Answer 1849

Question 1850

Draw the causes of purpura or easy bruising

Answer 1850

Question 1851

Draw the causes of abnromal bleeding in a child

Answer 1851

Question 1852

Def: ITP

Answer 1852

**Isolated low platelet count** (\<150x10⁹) in the **absence of other causes** of thrombocytopenia May be accompanied by **compensatory increase in megakaryocytes** in the bone marrow **2-10 years old**

Question 1853

Short history of days/weeks 1-2 weeks after **viral infection** **Petechiae, purpura** and/or **superficial brusing** **Epistaxis, mucosal bleeding** **Profuse bleeding is UNCOMMON**

Answer 1853

ITP

Question 1854

Dx of ITP

Answer 1854

Dx of exclusion FBC Blood film Bone marrow examination

Question 1855

Mx of ITP

Answer 1855

80% is **self-limiting** Most do not need treatment (even if platelet \<10x10⁹/L) Only given if evidence of **major bleeding** (intracranial/GI haemorrhage) or **persistent minor bleeds** **Oral prednisolone, IV anti-D, IV immunoglobulins** **Advice:** avoid trauma/contact sports

Question 1856

Def: chronic ITP

Answer 1856

Platelets remain low 6m after the Dx

Question 1857

Mx of chronic ITP

Answer 1857

_Chronic ITP_ (platelets remain low 6 months after diagnosis) **SUPPORTIVE** Drug treatment given only with _persistent bleeds_: **Rituximab** (anti-CD20 mAb) **Thrombopoietic growth factors** **Splenectomy** (those who fail drug therapy) Screen regularly for SLE as it may predate development of autoantibodies

Question 1858

Which type of haemophilia is more comon?

Answer 1858

Haemophilia A

Question 1859

Cx of the ahemophilias

Answer 1859

**Arthritis** if bleeds not controlled _Complications of treatment_ **Inhibitors/antibody** formation to FVIII or FIX Transfusion transmitted **infection** (Hep A,B,C, HIV) **Vascular access** – central venous access à infection, thrombosis

Question 1860

**intracranial haemorrhage, bleeding post-circumcision** or **prolonged bleeding from heel stick/venepuncture** **Haemarthroses** **Large haematomas**

Answer 1860

?Haemophilia

Question 1861

Ix in haemophilia

Answer 1861

Detailed FHx Analysis of coagulation factors

Question 1862

Primary care mx of haemophilia

Answer 1862

Home treatment – replacement therapy **Prophylactic factor replacement** for severe haemophilia **Desmopressin** for mild haemophilia A (no need for blood products) àstimulates FVIII and vWF release

Question 1863

Severity of haemophilia: Mild

Answer 1863

Bleeds after surgery

Question 1864

Severity of haemophilia: Moderate

Answer 1864

Bleeds after minor trauma

Question 1865

Severity of haemophilia: Severe

Answer 1865

Recurrent, spontaenous, muscle bleeds

Question 1866

Secondary Mx of haemophilia

Answer 1866

Prompt **IV infusion of recombinant FVIII/IX concentrate** If recombinant unavailable, highly purified, virally inactivated plasma-derived products Raising circulating factor level to **~30%** **normal is sufficient** to treat **minor bleeds/simple joint bleeds** **Major/life threatening bleeds** require **100% -** maintained 30% for 2 weeks to **prevent 2° haemorrhage** (regular/continuous infusion) Intramuscular injections, aspirin, NSAIDs AVOIDED AT ALL COST #

Question 1867

Def: vWD

Answer 1867

Quantitative or qualitative **deficiency of von Willebrand Factor (vWF)** Causing à **defective platelet plug formation** à **deficiency in FVIII:C** Different subtypes but inheritance is usually **autosomal dominant** Commonest subtype, **Type 1**, is **fairly mild** and is often not diagnosed until **puberty/adulthood**

Question 1868

**Mucosal bleeding** **à Epistaxis** and **menorrhagia** **Bruising** **Excessive, prolonged bleeding after surgery** **Family history** of bleeding Spontaneous, soft tissue bleeding are UNCOMMON

Answer 1868

vWD

Question 1869

Ix in vWD

Answer 1869

Prothrombin time (PT) Activated partial thromboplastin time (APTT) RBC vWF antigen vWF function assay (ristocetin cofactor and collagen binding assays) Factor VIII activity _Consider:_ vWF multimer analysis, platelet aggregometry

Question 1870

Mx of vWD

Answer 1870

_Mild disease/Type 1 vWD_ **DDAVP** which causes endogenous release of FVIII and vWF Use with caution \<1 years as it can cause **hyponatremia, seizures** _Severe disease_ **_Plasma-derived_ FVIII concentrate** (recombinant FVIII would not contain vWF) Intramuscular injections, aspirin, NSAIDs AVOIDED AT ALL COST #

Question 1871

Paediatric HIV classification: Category N

Answer 1871

Asymptomatic

Question 1872

Paediatric HIV classification: Category A

Answer 1872

Mild immunosuppression: \>2 of lymphadenopathy, hepatomegaly, splenomegaly, parotitis, dermatitis, recurrent URTI/sinusitis/otitis media

Question 1873

Paediatric HIV classification: Category B

Answer 1873

Moderate immunosuppression: Bacterial meningitis/pneumonia/sepsis (1 epsidoe) Oropharyngeal candidiasis \>2m duration Recurrent/chronic diarrhoea Lymphocytic interstitial pneumonitis Purpura due to thrombocytopenia Nephropathy Disseminated varicella Fever \>1m

Question 1874

Paediatric HIV classification: Category C

Answer 1874

* severe AIDS-defining illness: opportunistic infections (Pneumocystus carinii pneumonia), severe failure to thrive, encephalopathy (may present with developmental delay), malignancy * children with SPUR infections (serious, persistent, unusual, recurrent) should be tested for HIV may present with complications of vaccination (eg: disseminated BCG)

Question 1875

Secondary causes of immunodeficiency

Answer 1875

HIV, intercurrent bacterial/viral infection, malignancy, malnutirtion, immunosuppression, nephrotic syndrome haematological disorders causing hyposplenism e.g. SCD TB, CF, EBV Neglect Malabsorption metabolic disease

Question 1876

Dx of HIV \<18m

Answer 1876

HIV DNA PCR is used for diagnosis. Positive maternal IgG HIV antibodies only indicate exposure but not infection

Question 1877

Dx of HIV \>18m

Question 1878

Crtieria for child being non-HIV infected:

Answer 1878

* Infants should be tested at 1 day, 6 weeks and 12 weeks of age. If all these tests are negative and the baby is not being breastfed, the parents can be informed that the child is not HIV-infected A confirmatory HIV antibody test is performed at 18 months of age.

Question 1879

Mx of HIV in pregnancy

Answer 1879

HAART for mother C section Avoid breast feeding

Question 1880

Mx of infants born to HIV-infected mothers?

Answer 1880

Test for HIV Postnatal ART chemoprophylaxis within 4 hours of birth Monotherapy of zidovudine normally given, if high risk of HIV start HAART

Question 1881

HAART in children

Answer 1881

Initiate in HIV positive children \<1 because infants have a higher risk of disease progression

Question 1882

Mx of children with confirmed HIV infection

Answer 1882

Specialist ID paediatrician Refer to PENTA guidelines: he guidelines recommend that ART should be started: In all HIV-infected children under 1 year of age. In all children with significant disease (WHO stage 3 or 4 or CDC stage B or C). In asymptomatic children over 1 year of age based on age-specific CD4 count thresholds (as detailed in the PENTA guidelines). Before the CD4 count reaches the CD4 treatment threshold. In those with hepatitis C virus or active TB co-infection. Other possible indications are: Asymptomatic children over 5 years at CD4 counts of 350-500 cells/μl, to potentially optimize CD4 count in adulthood. Children with a high viral load (\>100,000 copies/mL). Asymptomatic children aged 1-3 years irrespective of immune status and viral load. Sexually active adolescents, to minimise the risk of onward transmission. Significant HIV-related clinical symptoms. Hepatitis B virus co-infection irrespective of immune status.

Question 1883

Infection prophylaxis in HIV-positive infants

Answer 1883

Receive co-trimoxazole against PCP regardless of CD4 count After that its use depends on specific CD5 count

Question 1884

LT management of HIV infected children

Answer 1884

* Ix and tx co-morbid infx eg: hepatitis, EBV, CMV, HSV, MMR, toxoplasmosis, malaria film, mantoux test * Monitor CD4 counts, viral load, regular screening Other issues to consider: Assess compliance, disclosure of HIV diagnosis, future planning for child, planning for future pregnancies

Question 1885

Limbs the most common site Persistent localised bone pain – more severe at night and imporves with NSAID Sweeling Poss pathological # Otherwise well

Answer 1885

Osteogenic sarcoma

Question 1886

How can causes of developmental delay be classified?

Answer 1886

Prenatal Perinatal Postnatal

Question 1887

Answer 1887

Question 1888

Def: delay

Answer 1888

Slow acquisition of skill

Question 1889

Def: LD

Answer 1889

In relation to children of school age- cognitive, physical, specifical funcitonal skills

Question 1890

Def: disorder

Answer 1890

Maldevelopment of skill

Question 1891

Def: impairment

Answer 1891

Loss/abnromality of normal physiological funciton/structure

Question 1892

Def: disability

Answer 1892

Restriction/lack of ability due to impariment

Question 1893

Ex in developmental delay

Answer 1893

FHx Obstetric hx Developmental hx Trauma and infeciton School reports Regression Assess risk of NIA/neglect O/E: Look for growth patterns Test all 4 domains of development Look for dysmorphic features Neurological examinatoin CVS exam for abnormalities linked to dysmorphic syndromes Visual funciton and ocular abnormalities Hearing Patterns of mobility hand dominance etc Cognition

Question 1894

Ix in developmental delay

Answer 1894

**Cytogenic** – karyotyping, fragile X analysis or FISH analysis **Metaboli** – TFT, LFT, bone chem, U/Es, special tests for inborn errors I**nfection**- congenital infection screen (TORCH: Toxo, Other (syphilis), Rubella, CMV, Herpes) **Imaging** – Cranial USS in newborn, CT/MRI briain, skeletal survey, boen age **Histopath** – nerve, muscle biopsy **Others** – hearing, vision, cognition, therapy/psych/dietician/school assessment

Question 1895

Def: cerebal palsy

Answer 1895

Abnormality of movement and posture causing activity limitation attributed to non-progressive disturbances (lesions non-progressive but clinical manifestations emerge over time, motor disorder can evolve) that occurred in the developing fetal or infant brain. Accompanied by disturbances in other areas too. 2/1000 live births \*Term used until age up to 2years, after that – acquired brian injury is the proper term

Question 1896

Aetiology of CP

Answer 1896

**80% antenatal**: vascular occlusion, congenital infection, genetic disorder, cerebral dysgenesis **10% perinatal**: birth asphyxia, metabolic disorder **10% postnatal**: injury/trauma, infexn-encephalitis/meningitis, hypoglycaemia, hydrocephalus, hyperbilirubinaemia (kernicterus), brain abcess, space-ocucpying lesion, cyanotic heart disease à thrombi

Question 1897

* abnormal posture, tone in limb and trunk * delayed motor milestones * slowing of head growth * feeding difficulties * abnormal gait * asymmetric hand function/showing limb dominance before 12 months (kids don’t show this till later so if present pathology) * primitive reflexes persists stop development from progressing

Answer 1897

Early features of CP

Question 1898

How can CP be classified

Answer 1898

Spastic Dyskinetic Ataxic/hpotonic

Question 1899

Early trunk and limb hypotnoia, poor balance, delayed motor milestones à intention tremor, incoordinate movemnts, ataxic gait. Genetically determined but if acquired injury to cerebellum- same side as lesion, but can be relatively symmetrical

Answer 1899

Ataxic CP

Question 1900

intellect unimpaired, floppy/poor trunk control, delayed motor development à involuntary movements at end of 1^st year of life – uncontrolled, stereotyped; more evident with active movement/stress. Eg. **Chorea** – irregular, sudden, brief and non-repetitive; **athetosis** – slow writhing movement, more distally; **dystonia** – simultaneous contraction of agonist and antagonist muscles of trunk and prox muscles à twisting. Dmg/dysfnx of basal ganglia, extrapyramidal pathways – most commonly due to HIE DDx of uncontrollable momvements= kernicterus, Wilson’s, anti-emetics (pehnothiazines,metoclorpromaide, prochlorperazine), strep (grp A β-haemolytic)

Answer 1900

Dyskinetic CP

Question 1901

damage is to UMN (pyramidal or corticospinal tract) à increased muscle tone (velocity dependent, fast stretched = greater resistance à dynamic catch!!! à resistance will yield under pressure – clasp-knife) * **hemiplegia –** unilateral involvement of arm and leg, arm\>leg. Present at 4-12months, flaccid and hypotonic initially à spasticity develops. PMHs can be unremarkable – some neonatal stroke à larger lesions = hemianopia * **quadriplegia:** all 4 limbs, trunk extensor posturing (opisothonus), poor head control and low central tone. Seizures, microcephaly, moderate-sev IQ impairement. Perinatal HIE * **diplegia:** all 4 limbs but legs \>\> arms, hand fxn looks relatively normal. Abnormal gait. Associated with preterm birth due to periventricular brain dmg (PVL/IVH)

Answer 1901

Spastic cerebal palsy

Question 1902

Ix in CP

Answer 1902

MRI useful to confirm but unnecessary Clinical diagnosis by observation and testing of movements (with therapist, use of toys) If isolated motor delay – CPK/CK to rule out muscle dystrophy

Question 1903

Mx of CP

Answer 1903

Refer to neurodevelopmental disability clinic * Give parents diagnosis and information early – prognosis hard to say Wide range of associated medical, psych and social problems so MDT is v impt * **Specialist health visitor** = coordiates MDT, advice on development of play, local authority schemes * **Dietician** * **Social worker/services**: benefits – disability, mobility, housing, respite care, support agencies for patient and for cares; day nursery placements, advocate for child/family, statemetning and register of child with special needs * **Clinical psychologist & educational psychologist**= cognitive testing, educational advice, behaviour mgmt. * **Paediatrician** = Assessment, ix and dx, continue medical mgmt., coordinate input from therapists/other agenice * **SALT**= feeding, lang devep * **OT**= hand eye coordination, ADL, housing adaptations * **PT** = balance nad mobility, posture maintenance, mobility aids/orthoses, prevent contractures/spinal deformities **GP** =

Question 1904

Def: Abnormal speech and language development

Answer 1904

Can be receptive , expressive speech & language or both. Can be in technicalities of speech production – stammering, dysarthria, verbal dyspraxia; or in understanding meaning – inability/diffulty producing when he knows what is needed to be said, pragmatics, semantics, social/comm skills

Question 1905

Ix in speech and language problems

Answer 1905

* In history-is this a speech or language problem? Speech disorder- words unintelligible, but child can comprehend vs if both comprehension and speech language difficulty. FHx, when parents have noticed this, birth Hx, other parts of development, can the kid hear (when you call out his name does he respond? PMHx- ear infexns etc * O/E – look into the ears – otitis media? * Look at other areas of development (in early years- large overlap with IQ development), autism has problems with social/interpersonal problems , LD other areas affected too * Hearing test * SALT assessment Test of language development: Symbolic toy test – early language development and Reynell test for receptive and expressive language – pre-schoolers

Question 1906

Def: hearing impairment

Answer 1906

Sensorineural: May be profound (\>95dB hearing loss) progressive genetic, congenital infection, prem, HIE, hyperbili, meningitis/encephalitis, head injury, drugs (aminoglycosides, frusemide, neurodegerative disorders Conductive: Max 60dB hearing loss Glue ear, Eustachian tube dysfunction (Downs, Cleft palate, Pierre Robin sequence, Mild facial hypoplasia), wax

Question 1907

Low intelligence thresholds

Answer 1907

Borderline and Mild – IQ 70-80 Moderate – IQ 50-70 Severe – IQ 35-50 Profound – IQ \<35

Question 1908

When is mild LD identified?

Answer 1908

When child starts school

Question 1909

Features of dyspraxia

Answer 1909

* Disorder of motor planning +/- execution with no significant findings on standard neuro examination. Disorder of higher cortical processes assoc/ problems of perception, use of language and putting thoughts together * Features: problems with… * Cutting up food * Poorly established laterality * Copying and drawing * Messy eating, dribbling * If mild, undetected during the first few years of life * Ix: OT, SALT assessment, visual assessment * Tx: therapy (sensory integration ,sequencing and executive planning) * Improve with therapy and maturation

Question 1910

Def: dyslexia

Answer 1910

Child's reading age is \>2y behind chronological age

Question 1911

Draw the causes of developmental delay

Answer 1911

Question 1912

Draw the classificaiton of hearing difficulties in children

Answer 1912

Question 1913

For what is DS a risk in terms of hearing?

Answer 1913

Congenital conductive deafness

Question 1914

What are the hearing tests that can be used in older children

Answer 1914

Distraction hearing test Speech discrimination test Visual reinforcement audiometry Impedance audiometry tests (test if middle ear is functioning)

Question 1915

Mx of sensorinueral hearing impairment

Answer 1915

Sensorineural hearing impairment – need early amplification with hearing aids for speech and language development à if this gives insufficient amplification, cochlear implants required

Question 1916

* loss of red reflex due to cataract * white reflex – retinoblastoma, cataract, retinopathy of prematurity * not smiling by 6 weeks * lack of eye contact * visual inattention * random eye movements * * squint = strabismus -\> may have FH photophobia

Answer 1916

?Visual defect

Question 1917

Draw classification of causes of visual impairment

Answer 1917

Question 1918

Def: LD

Answer 1918

Learning disability = Significant impairment of all 3 of: 1. intellectual functioning (IQ\<70) 2. social or adaptive functioning 3. both of these impairments present before 18 years old

Question 1919

Cause of moderate,severe, profound LD

Answer 1919

Usually organic: brain damage genetic abnormalities hypothyroidism

Question 1920

What are the genetic causes of LD

Answer 1920

DS Fragile X PWS Angelman

Question 1921

What is the commonest genetic cause of LD

Answer 1921

DS

Question 1922

Ix LD

Answer 1922

Exclude organic causes – hearing and vision tests, TFT Intellectual impairment assessed by WAIS III = Wechsler Adult Intelligence Scale Adaptive/social functioning assessed by ABAS II = Adaptive Behaviour Assessment Scale Clinical interview and school reports

Question 1923

What tool can be used to assess intellectual impairment?

Answer 1923

WAISIII Weschler Adult Intelligence Scale

Question 1924

What can be used to assess adpative/social funcitoning?

Answer 1924

ABASII Adaptive behaviour assessment scale

Question 1925

Def: ASD

Answer 1925

Impairment of social interactions and social communication combines with restricted interest and rigid and repetitive behaviour If only some of the features are present then child is said to have autistic features and not the full spectrum Aperger syndrome- have social impairment of autism ( milder) but near normal speech development

Question 1926

Cx of ASDs

Answer 1926

General learning and attention difficulties Seizures- not until adolescence

Question 1927

What are the domains affected by ASD

Answer 1927

Impaired scoial interactions Speech and language disorders Ritualistic and repetitive behaviour

Question 1928

What are the features of impaired social interactions in ASD?

Answer 1928

* No close friendships, doesn’t seek comfort * Prefers own company * No interested in play * Gaze avoidance * Doesn’t appreciate others have though and feelings * Cannot appreciate social cues

Question 1929

What are the features of SAL in ASD?

Answer 1929

* Limited gestures and facial expressions * Monotonous voice * Over literal interpretation of speech * Formal pedantic language * Echoes questions, repeats instructions

Question 1930

What are the features of ritualisitc and repetitive behaviour in ASD

Answer 1930

* Violent tempers if disrupted * Tiptoe gait and hand flapping * No imagination in lay Peculiar interest and repetitive adherences

Question 1931

Mx of ASD

Answer 1931

Refer if ?autism Establish a team of healthcare professional swho are responsible for the care Lead clinician- generally paediatrician Treatment with: Applied behavioural analysis, requires 20-30h of therapy each week so time consuming and not often used but can Help reduce ritualistic behaviour Develop language Develop social skills Learn to play

Question 1932

Def: colic

Answer 1932

A common symptom complex that occurs during thye first few months of life marked by paroxysmal, uncontrollable crying in an otherwise healthy, well fed baby

Question 1933

Paroxysmal incosolable crying or screaming Drawing up of the kness Passing excessive flatus several times a day particularly in the evening

Answer 1933

?colic

Question 1934

Mx of colic

Answer 1934

Normally resolves by 4m Benign condition Support and reassurance of parents If severe and persistent, start to think about GORD or CMPA Empiricaly 2 week trial of whey hydrolysate formula, followed by antireflux trial

Question 1935

* Predominantly **negative** moods – whinging, moaning, crying * **Intense emotional reactions** – screaming rather than whimpering, jumping for joy rather than smiling * **Irregular biological functions** – a lack of rhythm in sleeping, hunger or toileting * **Negative initial responses** to **novel situations**, e.g. pushing a new toy away **Protracted adjustment to new situations** – taking weeks or months to settle into a new playgroup

Answer 1935

Difficult temperament NB this pattern is a vulnerability factor for future emotional and behavioural problems

Question 1936

What are the advanatges of breast feeding for the infant

Answer 1936

Ideal nutrition Lifes saving in developing countries Reduces the risk of GI infection and NEC in preterm Enhacnes realtionship Reduces risk of IDDM and HTN and obesity in later life

Question 1937

Advantages of breast feeding for the mother

Answer 1937

Promotes close attachment between mother and baby Increases the time interval between children Helps with a possible reduction in premenopausal breast cancer

Question 1938

What are the properties of breast milk that explain its advantages

Answer 1938

Anti-infective: Secretory IgA Bifidus factor: promotes lactobacillus bifidis Contains bacteriolytic enzymes and antiviral agents Cellular factors Easily digestible protein Good lipid quality Ca:P 2:!, prevents hypocalcaemic tetany and improces Ca absorption Low renal solute load Bioavailable Fe Contatins long chain poly-unsaturated acids that are important in retinal development

Question 1939

What are the potential complications of breast feeding?

Answer 1939

Unknown volume of milk Infection transmission Breast-milk jaundice Drug transmission Nutrient inadequacies Vit K deficiency Potential transmission of environmental contaminants Less flexible Potentially emotional upset if there are difficulties

Question 1940

How can breast feeding lead to nutrient inadequacies?

Answer 1940

Breast-feeding beyond 6m without timely introduction of appropriate solids may lead to poor weight gain and ricekst

Question 1941

Pain – back/joint/limb/genital pain CVS/resp – breathing difficulty, palpitations, chest pain Abdo – stomach pain, N + V, poor appetite CNS – headache, dizziness muscle weakness, tremor Pattern: recurrent, improves on weekends/school holidays, associated with non-attendance, presence of stressors

Answer 1941

Somatisation

Question 1942

When should an organic cause of ?somatisation be suspected?

Answer 1942

PAIN WAKING CHILD * family history of similar symptoms * unexplained fever * significant diarrhoea/vomiting * involuntary weight loss * poor growth * * raised ESR

Question 1943

Mx of somatisation?

Answer 1943

1. Education - good prognosis for resolution (50% improve) 2. Pain management – relaxation, distraction 3. Manage underlying factors – may have to reduce reinforcement by parents, normalize activities, non-pain based shared activities, reduce stressors and risk factors 4. Manage co-morbidities – anxiety/depression Referral to CAMHS if co-morbidities exist, persistent or suicidal If persistent (15% of children), family CBT may be necessary

Question 1944

Round face, flat nasal bridge Upslanted palpebral fissures Epicanthic folds Brushfield spots in iris Small mouth, protruding tongue Small ears Flat occiput and 3^rd fontanelle Short neck Single palmar creases, incurved 5^th finger and wide ‘sandal’ gap between toes hypotonia congenital heart defects (40%), duodenal atresia, Hirschsprung disease Later medical problems Delayed motor milestones Mod to severe learning difficulties Small stature ↑risk of infections

Answer 1944

DS

Question 1945

Mx of DS

Answer 1945

Early intervention: PT, SALT Counsel parents for assistance available – professional and family support groups (Downs Syndrome Association) Counsel also for assisting family with dealing with feelings of grief, anger, guilt etc Child Development Service – coordinate care * Regular review or development and health * SALT, physio, OT, dietician, GP, social worker, audiologist, ophthalmologist, paediatrician, cardiologist SEN, Specialist schools

Question 1946

Cx of Turners

Answer 1946

* Cardiac complications * ↑risk of AI conditions * HTN common * Renal abnormalities → recurrent UTI * Vision problems * Hearing impairment due to persistent ear infections Osteoporosis

Question 1947

Females In utero USS: oedema of the neck, hands or feet, cystic hygroma Live-born: lymphoedema of hands and feet in neonate (may persist) * Spoon shaped nails * Short stature * Neck webbing * Cubitus valgus * Widely spaced nipples * Congenital heart defects (esp coarctation of the aorta) * Delayed puberty * Ovarian dysgenesis → infertility (primary amenorrhoea) * * Renal anomalies * Pigmented moles * Recurrent otitis media * Normal intellect in most

Answer 1947

Turner

Question 1948

Mx of Turners

Answer 1948

GH therapy Oestrogen replacement

Question 1949

Def: Noonans snydorome

Answer 1949

Turner-like syndrome in males Cauised by AD mutation, normal karyotype

Question 1950

In utero: polyhydraminos, pleural effusions, oedema, increased nuchal fluid with normal karyotype * Characteristic facies – more apparent during childhood (low set ears, ptosis, anti-mongoliod palpebral slant, wide spacing of eyes) * Occasional mild learning difficulties * Shorted webbed neck with trident hair line * Pectus excavatum * Congenital heart disease (esp. pulmonary stenosis, atrial septal defect) * Scoliosis, joint laxity, cubitus valugus Short stature (80%)

Answer 1950

Noonans

Question 1951

Mx of Turners and Noonans

Answer 1951

GH therapy (oestrogen development) Surgery for cardiac abnormalities Involvement of dentist Genetic counselling

Question 1952

* After short period of normality, severe neonatal illness with poor feeding, vomiting, encephalopathy, acidosis, coma and death Infant/older child – similar to above but hypoglycaemia a prominent feature or as Acute life-threatening episode, or near-miss cot-death Subacute: period of normal development with regression, organomegaly and coarse facies

Answer 1952

?Metabolic disorders

Question 1953

* Facial abnormalities (Microcephaly, Flat philtrum, Thin upper lip, Retrognathia in infancy, micrognathia or relative prognathism in adolescence and a low nasal bridge, Microphthalmia, [strabismus](http://www.patient.co.uk/doctor/Squints.htm), [ptosis](http://www.patient.co.uk/doctor/ptosis-and-lid-lag) and short palpebral fissures [Cleft palate](http://www.patient.co.uk/doctor/Cleft-Lip-and-Palate.htm), Posterior rotation of the ears * IUGR, FTT * Neuro-developmental delay ADHD, memory problems, poor problem solving skills Poor coordination, speech and language delay, sucking and feeding problems in neonate * Congenital heart defects: ASD, VSD * Urogenital defects: cryptorchidism, hypoplastic labia * * Partial deafness, visual disability

Answer 1953

FAS

Question 1954

Low birthweight Prominent occiput Small mouth and chin Short sternum Flexed, overlapping fingers Rocker bottom feet Cardiac and renal malformations

Answer 1954

Efwards syndrome (trisomy 18)

Question 1955

Structural defect of brain Scalp defects Micropthalmia and other eye defects Cleft lip and palate Polydactyly Cardiac and renal malformations

Answer 1955

Patau (trisomy 13)

Question 1956

Infertility Hypohonadisim Pubertal development may appear normal Gynaecomastia in adolescence Tall stature Intelligence usually in the normal range

Answer 1956

Klinefelter syndrome

Question 1957

Moderate-severe LD Macrocephaly Macro-orchidism Characteristic faces: postpubertal, large everted ears, prominent mandible and broad forehead Mitral valve prolapse, joint laxity, scoliosis, autism, hyperactivity

Answer 1957

Fragile X

Question 1958

Short stature Characteristic facies Trnaisnet neonatal hypercalcaemia CHD Mild to moderate learning difficulties

Answer 1958

Williams syndrome

Question 1959

Characteristic facies Hypotonia Neonatal feeding difficulties FTT Obesity later in childhood Hypogonadism Developmental delay LD

Answer 1959

PWS

Question 1960

Answer 1960

Edwards

Question 1961

Answer 1961

Patau

Question 1962

Answer 1962

Fragile X

Question 1963

Answer 1963

Noonan

Question 1964

Answer 1964

Williams syndrome

Question 1965

Answer 1965

PWS

Question 1966

What are the areas in which children need safeguarding

Answer 1966

Physical abuse Emotional abuse Sexual abuse Neglect Fabricated or induced illness

Question 1967

* Babies: apathetic, delayed development, non-demanding; described negatively by mother * Toddlers, pre-schoolers: violent, apathetic, fearful

Answer 1967

Emotional abuse

Question 1968

* Physical symptoms: vaginal bleed/itching/discharge, rectal bleed * Behavioural: soiling, secondary eneuresis, self-harm, aggressive/sexualised behaviour, regeression, poor school performance

Answer 1968

?sexual abuse

Question 1969

Def: neglect

Answer 1969

persistent failure to meet a child’s basic physical and/or psychological needs – likely to result in serious impairment of health or development. Failure to provide: food, clothing. Shelter, protection, supervision

Question 1970

* Child consistently misses medical appointments.school, lacks medical and dental care, seems ravenous, no glasses/immunisations when needed, inadequate clothing in winter, dirty, abusing substances, says no one is at home to care for them * Caregiver appears indifferent, apathetic or depressed. Behaves irrationally/bizzarely, is abusing etoh/drugs.

Answer 1970

?Neglect

Question 1971

What are the risk factors for abuse

Answer 1971

* In child: failure to meet parental expecations, resulted from unwanted pregnancy * Parent/carer: mental health problems, indifference, intolerance over-anxiousness, alcohol/drug abuse * Family: step-parents, domestic violence, multiple/closely spaced births, social isolation or lack of social support, young parental age * Environment: poverty, poor housing, dangerous neighbourhood

Question 1972

What are the red flags for NAI

Answer 1972

Injuries in very young children Explanations which do not match the appearance of injury and sound unconvincing Multiple tpyes and age of injury Injuries which are classic in site or character Delay in presentation Things the child may communicate during te evaluation

Question 1973

Bruises in toddlers

Answer 1973

Multiple brusises are commonly found on the legs of any toddler Brusises at other sites may be suspicious Pattern of the bruise may indicate how it was acquired

Question 1974

Burns/scalds in NAI

Answer 1974

When a toddler accidently scalds themselves the scald is usually irregular/asymmetrical Inflicted scalds are classically symmetrical e.g. donut shaped lesion on the buttocks where the bottom of the bath protects wthe skin from contact with hot water.

Question 1975

How to find hidden head injuries

Answer 1975

Examine the fundi for retinal haemorrhages which may occur when a baby is shaken and can be associated with the presence of SDHs

Question 1976

Examination for signs of sexual abuse

Answer 1976

Should only be carried out by an experienced paediatrician

Question 1977

Mx of NAI

Answer 1977

Thorough hx and examination Careful documentation Measure height weight etc Treat specific injuries If abuse suspected/confirmed, need to decide if the child needs immediate protection- admit to hospital as place of safety, legal enforcement may be required. May have to be placed in a foster home Immediately alert seniors, safeguarding team at hospital and follow local procedures. CHILDS SIBLINGS Police and social services Strategy meeting: Decide whether child needs child protection plan Whether there should be a court applicaiton to protect the child Whether F/U is necessary

Question 1978

What is the fraemwork to assess child safeguarding and to promote welfare

Answer 1978

Child's developmental needs Family and environemntal factors Parenting capacity

Question 1979

What are the mechanisms for immediate protection of a child

Answer 1979

Risk to the life or of serious, immediate harm: ACT IMMEDIATELY TO SECURE SAFETY OF CHILD If it is necessary to remove a child forom their home: Emergency Protection Order (police can do this without court approval as a last resort) Local authority is responsible for taking emergency action. Children Act can be used

Question 1980

What is the Section 47 of the Children Act

Answer 1980

Section 47 of the Children Act 1989 places a duty on LAs to investigate and make inquiries into the circumstances of children considered to be at risk of ‘significant harm’ and, where these inquiries indicate the need, to decide what action, if any, it may need to take to safeguard and promote the child’s welfare. The investigation will form a core assessment, which is an in-depth assessment of the nature of the child’s needs and the capacity of his or her parents to meet those needs within the wider family and community context. The results of that assessment will form part of the LA’s evidence if it commences proceedings for a Care or Supervision order.

Question 1981

Dx of laryngomalacia

Answer 1981

Flexible laryngoscopy by ENT in OPD

Question 1982

Omega shaped epiglottis or arytenoid cartilage

Answer 1982

Laryngomalacia

Question 1983

What tool can be used to assess croup and what are hte domains?

Answer 1983

Westley Clinical scoring system Inspiratory stridor Intercostal recession Air entry Cyanosis Level of consciousness

Question 1984

What patient group may receive prophylaxis vs bronchiolitis What is this?

Answer 1984

High risk infants e.g. O2 dependent survivors of prematuriy Palivizumab

Question 1985

What is the most common cause of clubbing in children?

Answer 1985

CF

Question 1986

What is the commonest cause of acute deterioration in chronic asthma?

Answer 1986

Poor adherence

Question 1987

What is the commonest cause of cyanosis in the newborn

Answer 1987

TGA

Question 1988

Answer 1988

Question 1989

Answer 1989

Question 1990

Answer 1990