paeds

Question 1

signs of moderate resp distress

Answer 1

Tachypnoea, Tachycardia, Nasal flaring, Use of accessory respiratory muscles, Intercostal and subcostal recession, Head retraction, Inability to feed

Question 2

signs of severe resp distress

Answer 2

Cyanosis, Tiring because of increased work of breathing, Reduced conscious level, Oxygen saturation <92% despite oxygen therapy

Question 3

stridor

Answer 3

= predominantly inspiratory = from extrathoracic airway obstruction in the trachea and larynx

Question 4

causes of stridor

Answer 4

Commonly caused by croup, also epiglottitis, inhaled foreign body

Question 5

wheeze

Answer 5

predominantly expiratory = from intrathoracic airway narrowing.

Question 6

causes of wheeze

Answer 6

Bronchiolitis, asthma

Question 7

causes of coryza

Answer 7

Causes: rhinoviruses, coronaviruses and RSV

Question 8

sx coryza

Answer 8

Nasal discharge, nasal blocking, cough

Question 9

pharyngitis

Answer 9

= pharynx and soft palate are inflamed, and local lymph nodes are enlarged and tender

Question 10

causes pharyngitis

Answer 10

adenoviruses, enteroviruses, rhinoviruses, In older children = group A -haemolytic streptococcus is common.

Question 11

tonsilitis

Answer 11

is a form of pharyngitis which includes intense inflammation of the tonsils, often with a purulent exudate

Question 12

causes tonsilitis

Answer 12

= group A -haem strep and the Epstein-Barr virus.

Question 13

features tonsilitis

Answer 13

Headache, apathy, abdo pain, white tonsillar exudate and cervical lymphadenopathy are more common with bacterial infection. It is not possible to clinically distinguish between viral and bacterial tonsillitis.

Question 14

mx tonsilitis

Answer 14

Antibiotics e.g. penicillin V or erythromycin (if penicillin allergy) are often prescribed for severe pharyngitis and tonsillitis even though only 1/3 are caused by bacteria (10 days).

Question 15

fever pain criteria

Answer 15

Fever in past 24 hours
Absence of cough or coryza
Symptom onset ≤3 days
Purulent tonsils
Severe tonsil inflammation

Question 16

fever pain score interpretation

Answer 16

A score of 0-1 =No antibiotics recommended.
A score of 2=Delayed antibiotic may be appropriate.
A score of 3 =Delayed antibiotic may be appropriate.
A score of 4 or more = Consider antibiotics if symptoms are severe or a short delayed prescribing strategy may be appropriate (48 hours)

Question 17

what is fever pain score for

Answer 17

determining if bacterial or viral pharyngitis/tonsilits

Question 18

what is centor criteria for?

Answer 18

Estimates probability that pharyngitis is streptococcal

Question 19

centor critieria

Answer 19

Tonsillar exudate.
Tender anterior cervical lymphadenopathy or lymphadenitis.
History of fever (over 38°C).
Absence of cough

Question 20

interpreting centor score

Answer 20

A score of 0, 1 or 2 is thought to be associated with a 3-17% likelihood of isolating streptococcus. A score of 3 or 4 is thought to be associated with a 32-56% likelihood of isolating streptococcus.

Question 21

cause whooping cough

Answer 21

bordatella pertussis

Question 22

features whooping cough

Answer 22

After a week of coryza, child develops spasmodic cough followed by a characteristic inspiratory whoop. Often worse at night and may lead to vomiting. During heavy coughing, child may go blue or red in the face, and mucus flows from the nose and mouth.

Question 23

diagnosing whooping cough

Answer 23

culture of organism on pernasal swab, marked lymphocytosis on blood film (>15x109/L)

Question 24

mx whooping cough

Answer 24

Clarithromycin if present within 21d

Question 25

cause pneumonia

Answer 25

Newborn = group B streptococcus, gram -ve enterocci and bacilli. Infants and young children=. RSV, Strep pneumoniae or H. influenzae. Children >5 yrs- Mycoplasma pneumoniae, Strep pneumoniae, Chlamydia pneumoniae

Question 26

features pneumpnia

Answer 26

Fever, cough, rapid breathing, Preceded by URTI, lethargy, poor feeding, ‘unwell’ child.
Examination: Tachypnoea , Nasal flaring, Chest indrawing, May be end-inspiratory coarse crackles

Question 27

diagnosing pneumonia

Answer 27

CXR, A nasopharyngeal aspirate

Question 28

when to admit to hospital with pneumonia

Answer 28

xygen sats <92%, Recurrent apnoea, Grunting, Inability to maintain adequate feed/fluid.

Question 29

mx pneumonia

Answer 29

oxygen for hypoxia and analgesia for pain, IV fluids if necessary. Newborns = broad spectrum antibiotics. Older infants = oral amoxicillin, with broader spectrum e.g. co-amoxiclav reserved for complicated cases, >5 years = amoxicillin or oral macrolide e.g. erythromycin.

Question 30

bronchiectasis

Answer 30

permanent dilatation of the bronchi. May be generalised or restricted to a lobe of the lung.

Question 31

causes bronchiectassi

Answer 31

Generalised may be due to cystic fibrosis, primary ciliary dyskinesia, immunodeficiency or chronic aspiration.
Focal is due to previous severe pneumonia, congenital lung abnormality, or obstruction by a foreign body.

Question 32

ix bronchiectasis

Answer 32

CT

Question 33

cause croup

Answer 33

Parainfluenza, rhinovirus, RSV and influenza,
typically occurs from 6 months to 6 years of age the peak incidence is the 2nd year of life, in autumn.

Question 34

features croup

Answer 34

are coryza and fever, followed by: Hoarseness ,A barking cough, Harsh stridor. The symptoms often start, and are worse, at night

Question 35

mx croup

Answer 35

1st line treatment = oral dexamethasone, oral prednisolone, or nebulized steroids (budesonide). These reduce the severity and duration of croup and are first line for croup causing recession at rest. They have been shows to reduce the need for hospitalisation. If severe: nebulised epinephrine (adrenaline) with oxygen by face mask provides rapid but transient improvement.

Question 36

what is bacterial tracheitis

Answer 36

pseudomembranous croup)

Question 37

features bacteria tracheitis

Answer 37

High fever appears very ill, rapidly progressive airways obstruction with copious thick airway secretions. caused by staph aureus.

Question 38

mx bacterial tracheitis

Answer 38

IV abx and intubation and ventilation if required

Question 39

cause acute epiglottitis

Answer 39

H. influenzae type b (Hib), Most common aged 1-6 years but affects all age groups.

Question 40

features acute epiglottitis

Answer 40

Onset of epiglottitis is usually very acute, with: High fever in a very ill, toxic-looking child, An intensely painful throat that prevents the child from speaking or swallowing; saliva drools down the chin, Soft inspiratory stridor,The child sitting immobile, upright, with an open mouth to optimise the airway, cough minimal or absent

Question 41

what not to do in acute epiglottitis

Answer 41

DO NOT lie the child down or examine the throat with a spatula or perform a lateral neck X-ray must not be undertaken as they can precipitate total airway obstruction + death.

Question 42

mx acute epiglottitis

Answer 42

Senior anaesthetist, paediatrician, ENT surgeon should be involved. Intubation. Once airway is secured, blood should be taken for culture and abx started e.g. cefuroxime. Tracheal tube removed after 24hrs, abx 3-5 days. Prophylaxis rifampicin is offered to close household contacts (H.influenzae infection).

Question 43

cause bronchiolitis

Answer 43

RSV is the main cause (80%), but it can also be cause by parainfluenza virus, rhinovirus, adenovirus, human metapneumovirus or influenza virus.

Question 44

features brochiolitis

Answer 44

Coryzal symptoms may precede a dry cough and increasing breathlessness.
Examination:Tachypnoea and tachycardia, Subcostal and intercostal recession, Hyperinflation of the chest, Sternum prominent, Fine end-respiratory crackles, High-pitched wheezes – expiratory > inspiratory

Question 45

ix brobchiolitis

Answer 45

Pulse oximetry, CXR and blood gases only indicated if resp. failure is suspected.

Question 46

when to admit with bronchiolitis

Answer 46

Sleep apnoea, Persistent oxygen sats <90% in air, Inadequate oral fluid intake (50-75% of usual volume), Severe respiratory distress – grunting, marked chest recession, or a resp. rate over 70 breaths/minute.

Question 47

mx bronchiolitis

Answer 47

Humidified oxygen via nasal cannula or a head box, assisted ventilation – CPAP (continuous positive airway pressure

Question 48

prevention bronchiolitis

Answer 48

A monoclonal antibody to RSV (palivizumab)

Question 49

inheritance CF

Answer 49

Autosomal recessive, carrier rate 1 in 25. Defective protein called the CF transmembrane conductance regulator (CFTR). The gene for CFTR is located on chromosome 7.

Question 50

pathophysiology CF

Answer 50

abnormal ion transport across epithelial cells. In the airway, this leads to reduction in the airway surface liquid and consequent impaired ciliary function and retention of mucopurulent secretions. Defective CFTR also causes dysregulation of inflammation and defence against infection. The pancreatic duct becomes blocked by thick secretions, leading to pancreatic enzyme deficiency and malabsorption.

Question 51

features CF in children

Answer 51

Newborn= Diagnosed through newborn screening, Meconium ileus. Infancy= Prolonged neonatal jaundice, Growth faltering, Recurrent chest infections, Malabsorption, steatorrhea. Young child = Bronchiectasis, Rectal prolapse, Nasal polyp, Sinusitis. Older child and adolescent= Allergic bronchopulmonary aspergillosis, Diabetes mellitus , Cirrhosis and portal hypertension, Distal intestinal obstruction (meconium ileus equivalent), Pneumothorax or recurrent haemoptysis, Sterility in males
On examination: Persistent, wet cough, productive of purulent sputum, Hyperinflation of the chest – air trapping, Coarse inspiratory crepitations, Expiratory wheeze, Finger clubbing

Question 52

diagnosis CF

Answer 52

THE SWEAT TEST = concentration of chloride in sweat is markedly elevated. Cl 60-125mmol/L in CF, compared to 10-40mmol/L (normal).

Question 53

mx CF

Answer 53

Children should have physiotherapy at least twice a day, aiming to clear the airways of secretions. Medication: Continuous prophylactic oral abx (flucloxacillin) are recommended, with additional rescue abx for any flare ups, Daily nebulised antipseudomonal abx = slow decline of lung function caused by chronic Pseudomonas infection. Bilateral sequential lung transplantation is the only therapeutic option for end-stage CF lung disease
:, Due to increasing chest infections, CF patients are segregated and advised not to socialise with other people with the disease,

Question 54

CF in teenagers and adults

Answer 54

CF in teenagers and adults: Diabetes mellitus – decreasing pancreatic endocrine function, Evidence of liver disease with hepatomegaly on palpation, abnormal LFTs or an abnormal US, Distal intestinal obstruction syndrome (meconium ileus equivalent) is usually cleared by a combination of oral laxative agents, Due to increasing chest infections, CF patients are segregated and advised not to socialise with other people with the disease, Males are virtually always infertile due to absence of the vas deferens.

Question 55

features viral induced wheeze

Answer 55

only wheezes when has a viral upper respiratory tract infection (URTI) and is symptom free inbetween episodes

Question 56

mx viral induced wheeze

Answer 56

Tx when symptoms, no prophylaxis. 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

Question 57

features moderate acute asthma

Answer 57

able to talk
oxygen sat s>92
peak flow >50%
RR: </= 40 for 2-5y, </=30 for 5-12 yr, </= 25 for 12-18y
HR: </= 140 for 2-5y, </= 125 for 5-12 yr, </= 110 for 12-18y

Question 58

features severe asthms

Answer 58

too breathless to talk
oxygen sats <92
peak flow 33-50%
RR: > 40 for 2-5y, >30 for 5-12 yr, > 25 for 12-18y
HR: > 140 for 2-5y, > 125 for 5-12 yr, > 110 for 12-18y

Question 59

features life threatening asthma

Answer 59

silent chest, cyanosis
poor resp effort
exhasution
arrhythmia, hypotension
altered consciousness
agitation, confusion
oxygen sats <92

Question 60

mx moderate acute asthma

Answer 60

calm and reassure
SABA via spacer + face mask <3 2-4 puffs increaseing by 2 puffs every 2mins until 10 puffs if needed
oral pred 1-2mg/kg max 40mg
monitor response for 15-30min

Question 61

mx severe acute asthma

Answer 61

high flow oxygen
SABA via spacer, 10 puffs or nebulised (2.5mg salbutamol <8, 5mg >8)
oral pred or IV hydrocortisonw
consider; inhaled ipratropium, IV B agonist (salbutamol) or aminophylline or magnesium

Question 62

mx life threatening acute asthma

Answer 62

high flow oxygen
SABA via spacer, 10 puffs or nebulised (2.5mg salbutamol <8, 5mg >8)
oral pred or IV hydrocortisonw
nebulised ipratropium
consider; inhaled ipratropium, IV B agonist (salbutamol) or aminophylline or magnesium
discuss with PICU

Question 63

next steps if respondin tto tx in acutr asthma

Answer 63

responding: bronchodilators 1-4hr prn, discharge with 3-7d oral pred when on 4hrly
at discharge: rev medication and inhaler techique, personalsied action plan, arrange follow up

Question 64

steps if not responding to tx in acute asthma

Answer 64

transfer to HDU
senior rev
consider IV therapies not used (magnesium, aminophylline, B2agonist)
CXR and blood gas
consider mechanical venitaltion

Question 65

patterns of wheezing in asthma

Answer 65

Viral episodic wheezing – wheeze only in response to viral infections. Multiple trigger wheeze – in response to multiple triggers and which is more likely to develop into asthma over time, Asthma

Question 66

multiple trigger wheeze and asthma

Answer 66

Frequent wheeze can be triggered by many stimuli, such as: Viruses, Cold air, Dust, Animal dander, Exercise. Atopic asthma is strongly associated with other atopic diseases such as eczema, rhinoconjunctivitis and food allergy

Question 67

causes of persistent childhood wheeze

Answer 67

Viral episodic wheeze, Multiple trigger wheeze, Asthma , Recurrent anaphylaxis (e.g. in food allergy), Chronic aspiration, Cystic fibrosis, Bronchopulmonary dysplasia, Bronchiolitis obliterans, Trachea-bronchomalacia

Question 68

pathophysiology asthma

Answer 68

Atopy
Bronchial inflammation – oedema, excessive mucus production, infiltration with cells
Genetic predisposition
Environmental triggers e.g. URTIs, allergens, smoking, cold air, chemical irritants, emotional upset/anxiety, exercise
Bronchial hyperresponsiveness – exaggerated twitchiness to inhaled stimuli
Airway narrowing – reversible airflow obstruction (e.g. peak flow variability)
Symptoms – wheeze, cough, breathlessness, chest tightness

Question 69

features asthma

Answer 69

Clinical features: Asthmatic wheeze is a polyphonic, Symptoms worse at night and in the early morning, Symptoms that have non-viral triggers, Interval symptoms, i.e. symptoms between acute exacerbations, Personal or family history of an atopic disease, Positive response to asthma therapy
On examination: Long standing asthma – hyperinflation, generalised polyphonic expiratory wheeze with a prolonged expiratory phase. Onset of asthma in early childhood may result in Harrison’s sulci

Question 70

ix asthma

Answer 70

Younger children – asthma usually diagnosed from history and exam alone. Skin-prick testing for common allergens may be performed to aid the diagnosis of atopy and to identify allergens. Peak expiratory flow rate (PEFR) may be measured or spirometry performed - Poorly controlled asthma leads to increased variability in peak flow, with both diurnal and day-to-day variation.

Question 71

mx chronic asthma 5-16

Answer 71

1. SABA, 2. SABA + ICS, 3. SABA + ICS + LTRA, 4. SABA + MART, 5. SABA and MART with moderate dose ICS, 6. Asthma specialist: MART with high dose ICS or additional drug e.g. theophylline

Question 72

mx chronic asthma <5

Answer 72

1. SABA, 2. 8wk trial moderate dose ICS + SABA, 3. If sx recur within 4wk stopping ICS start low dose ICS, if after 4wk then redo trial, 4. ICS + LTRA + SABA, 5. stop LTRA and refer

Question 73

cause acute otitis media

Answer 73

Most common at 6-12 months of age. Infants + young children prone to it = Eustachian tubes are short, horizontal and function poorly.

Pathogens include viruses (esp. RSV and rhinovirus), bacteria incl. pneumococcus, H. influenzae and Moraxella catarrhalis

Question 74

sx acute otitis media

Answer 74

Pain in the ear + fever.

Question 75

complications acute otitis media

Answer 75

mastoiditis and meningitis (uncommon)

Question 76

mx acute otitis media

Answer 76

: analgesia. Most cases resolve spontaneously. Antibiotics reduce duration of pain, give if unwell still after 2-3d – amoxicillin usually

Question 77

glue ear

Answer 77

acute otitis media with effusion

Question 78

cause glue ear

Answer 78

Recurrent ear infections

Question 79

features glue ear

Answer 79

Usually asymptomatic apart from possible decreased hearing.
The eardrum is seen to be dull and retracted, often with a fluid level visible.

Question 80

mx glue ear

Answer 80

Conservation – do nothing, Eustachian tube auto inflation (Otovent balloon), In children that develop conductive hearing loss due to this, insertion of grommets (ventilation tubes) is performed, but benefits only last 12 months

Question 81

sensorineural hearing loss

Answer 81

caused by a lesion in the cochlea or auditory nerve and is usually present at birth

Question 82

conductive hearing loss

Answer 82

from abnormalities of the ear canal of the middle ear, most often from otitis media with effusion

Question 83

causes sensorineural hearing loss

Answer 83

Causes: Genetic (the majority). Antenatal and perinatal: Congenital infection, preterm, Hypoxic-ischaemic encephalopathy, Hyperbilirubinaemia. Postnatal: Meningitis/ encephalitis, Head injury, Drugs e.g. aminoglycosides, furosemide, Neurodegenerative disorders

Question 84

mx sensorienural hearin gloss

Answer 84

severe bilateral sensorineural hearing impairment will need early amplification with hearing aids for optimal speech and language development.
Children with microtia and meatal atresia can be helped with bone conduction hearing aids
Cochlear implants may be required where hearing aids give insufficient amplification
Gesture, visual context and lip movement will also allow children to develop language concepts.
Specialist teaching and support service in preschool and school years is provided by peripatetic teachers for children with hearing impairment.

Question 85

causes conducitve eharing loss

Answer 85

It is much more common than sensorineural hearing loss. In association with URTI, many children have episodes of hearing loss, which are usually self-limiting. In some cases of chronic otitis media with effusion, the hearing loss may last months or years.
Children with Down syndrome, cleft palate and atopy are particularly prone to hearing loss from middle ear disease.

Question 86

diagnosis conductive hearin g;oss

Answer 86

Impedance auditory tests, which measure the air pressure within the middle ear and the compliance of the tympanic membrane

Question 87

mx conductive hearin gloss

Answer 87

t: If the condition does not improve spontaneously, medical treatment (decongestant or a long course of abx or treatment of nasal allergy) can be given. If that fails, surgery is considered, with insertion of tympanostomy tubes (grommets) with or without the removal of adenoids.

Question 88

nystagmus

Answer 88

= A repetitive, involuntary, rhythmical eye movement.

Question 89

causes nystagmus

Answer 89

May be found in association with a structural eye problem (sensory defect nystagmus) but can also be a consequence of a problem at the cortical level.
If no structural eye or brain problem is found = idiopathic nystagmus.

Question 90

squint

Answer 90

strabismus) = Misalignment of the visual axes.

Question 91

causes strabismus

Answer 91

common underlying cause is refractive error, but cataracts, retinoblastoma, and other intraocular causes must be excluded. Higher incidence of strabismus in infants with cerebral palsy

Question 92

esotropia

Answer 92

One eye looks straight ahead; another eye looks towards the nose (convergent)

Question 93

exotropia

Answer 93

One eye looks straight ahead; another eye looks outwards (divergent)

Question 94

hypertropia

Answer 94

One eye looks straight ahead; another eye looks upwards (vertical)

Question 95

hypotropia

Answer 95

One eye looks straight ahead; another eye looks downwards (vertical)

Question 96

concomitant squint

Answer 96

non-paralytic, common) – usually due to a refractive error in one or both eyes. Correction of the refractive error with glasses often corrects the squint. The squinting eye most often turns inwards (convergent) but there can be outward (divergent), or vertical deviation

Question 97

paralytic squint

Answer 97

(rare) – varies with gaze direction due to paralysis of the motor nerves. This can be sinister because of the possibility of an underlying space-occupying lesion such as a brain tumour.

Question 98

ix squint

Answer 98

corneal light reflex, cover test

Question 99

mx squint

Answer 99

Conservative=Optical - glasses/ CL, Prisms, Orthoptic exercises. Surgery. Botulinum toxin injection

Question 100

causes severe visual impairment

Answer 100

Cataract, Albinism, Retinal dystrophy, Retinoblastoma, Congenital infection
Retinopathy of prematurity, Hypoxic-ischaemic encephalopathy, Cerebral abnormality/ damage, Optic nerve hypoplasia , Trauma, Infection , Juvenile idiopathic arthritis

Question 101

hypermetropia

Answer 101

(long sight): Can be corrected with convex (plus) lenses. These make the eye look bigger.

Question 102

myopia

Answer 102

Myopia (short sight): This is relatively uncommon in young children, presenting usually in adolescence. Can be corrected with concave (minus) lenses. These make the eye look smaller

Question 103

astigmatism

Answer 103

abnormal corneal curvature)

Question 104

amblyopia

Answer 104

clear image. Types: strabismic amblyopia, anisometropia amblyopia, ametropic amblyopia, meridional amblyopia, stimulus deprivation amblyopia

Question 105

tx refractive visual error

Answer 105

underlying condition, together with patching of the ‘good’ eye for specific periods of the day to force the ‘lazy’ eye to work and develop better vision

Question 106

sx preseptal cellulitis

Answer 106

: erythema and oedema around one eye but normal visual acuity and eye movements

Question 107

sx orbital celluliits

Answer 107

erythema and oedema around one eye, fever, reduced visual acuity, and painful eye movements – visual symptoms and limited eye movements

Question 108

mx orbital cellulitis

Answer 108

Need CT scan and IV abx

Question 109

sx bacterial conjunctivitis

Answer 109

itchy, irritated eyes with purulent discharge

Question 110

when does squint become abnormal

Answer 110

> 3m

Question 111

atrial septal defect

Answer 111

L to R shunt
Secundum ASD (80%)
Defect in the centre of the atrial septum involving the foramen ovale
Partial AVSD (common in trisomy 21)
Defect of the atrioventricular septum

Question 112

features atrial septal defect

Answer 112

Recurrent chest infections, arrhythmias, Ejection systolic murmur at the upper left sternal edge
CXR: Cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings
ECG: Secundum – RBBB, right axis deviation
Partial – superior QRS axis

Question 113

mx atrial septal defect

Answer 113

Secundum – cardiac catherisation with insertion of occlusion device
Partial – surgical correction

Question 114

VSD

Answer 114

L to R shunt
Small – smaller than the aortic valve
Large – same size/larger than the aortic valve

Question 115

features VSD

Answer 115

Small – loud pansystolic murmur, quiet P2
Large – soft panstolic murmur, thrill, loud P2
Heart failure, breathlessness, faltering growth, recurrent chest infections
CXR: Cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema
ECG: Biventricular hypertrophy

Question 116

mx VSD

Answer 116

Surgery at 3-6 months to prevent Eisenmenger’s

Question 117

PDA

Answer 117

L to R shunt
Presents in pre-term, flow of blood is from aorta to pulmonary artery

Question 118

features PDA

Answer 118

Continuous murmur, collapsing pulse, left subclavicular thrill, heaving apex bear, wide pulse pressure
Hepatomegaly, oedema

Question 119

mx PDA

Answer 119

Cardiac catherisation + insertion of occlusion device 1 yrs. Indomethacin to neonate

Question 120

tetralogy of fallot

Answer 120

R to L shunt
Large ventricular septal defect, aorta overriding the VSD, pulmonary stenosis, right ventricular hypertrophy

Question 121

sx and signs tetralogy of fallot

Answer 121

Ejection systolic murmur at the left sternal edge, clubbing, central cyanosis
Dyspnoea on exertion, delayed puberty, low growth, cyanosis within first wk
CXR: Boot shaped heart
ECG: RVH

Question 122

mx tetralogy of fallot

Answer 122

Surgery in first 2 years of life – closure of the VSD and correction of the pulmonary stenosis

Question 123

transposition of the great ateries

Answer 123

R to L shunt
Aorta is connected to the right ventricle and pulmonary artery is connected to the left ventricle. Blue blood returned to body; pink blood to lungs

Question 124

featuers transposition of the great arteries

Answer 124

Cyanosis by day 2 of life, loud/single S2, no murmur
CXR: Narrow upper mediastinum with an ‘egg on side’ appearance of the cardiac shadow
ECG: Biventricular hypertrophy

Question 125

mx transposition of the great arteries

Answer 125

Maintain ductal patency with prostaglandin infusion.
Balloon atrial septostomy

Question 126

AVSD

Answer 126

Seen in Down’s syndrome. A defect in the middle of the heart with a single five-leaflet valve between the atria and the ventricles. Stretches across the whole AV junction. Tends to leak

Question 127

features AVSD

Answer 127

Cyanosis at birth OR heart failure 2-3 weeks of life

Question 128

mx AVSD

Answer 128

Surgical repair 3-6 months of age

Question 129

aortic stenosis

Answer 129

Aortic valve leaflets are partly fused together, giving a restrictive exit from the left ventricle

Question 130

features aortic stenosis

Answer 130

Carotid thrill, ejection systolic murmur radiating to the neck, Only if severe stenosis – reduced exercise tolerance, chest pain on exertion, syncope
CXR: Prominent left ventricle with post stenotic dilatation of the ascending aorta
ECG: LVH

Question 131

mx aortic stenosis

Answer 131

Balloon valvotomy, aortic valve replacement

Question 132

pulmonary stenosis

Answer 132

Pulmonary valve leaflets are partly fused together, giving a restrictive exit from the right ventricle

Question 133

eatures pulmonary stenossi

Answer 133

Ejection systolic murmur, ejection click at upper left sternal edge. If severe – heave (right ventricle). Asymptomatic
CXR: Post stenotic dilatation of the pulmonary artery
ECG: RVH

Question 134

mx pulmonary stenossi

Answer 134

Transcatheter balloon diltation

Question 135

coarctation of the aorta

Answer 135

Congenital narrowing of the descending aorta - Excessive blood flow is diverted through the carotid and subclavian vessels into systemic vascular shunts to supply the rest of the body 🡪 stronger perfusion to the upper body than the lower

Question 136

features coarctation of the aorta

Answer 136

Radio-femoral delay, weak femoral pulse, systolic murmur, discrepancy between upper/lower limb BP. Heart failure
Can lead to infective endocarditis
CXR: cardiomegaly, irregularities of the inferior margins of the posteriorribs

Question 137

mx coarctation of the aorta

Answer 137

ABCs, prostaglandins, balloon dilation and surgery

Question 138

circulatory changes at birth

Answer 138

1.In the foetus, the left atrial pressure is low, as relatively little blood returns from the lungs.
2.The pressure in the right atrium is higher than in the left, as it receives all the systemic venous return including blood from the placenta.
3.The flap valve if the foramen ovale is held open, blood flows across the atrial septum into the left atrium, and then into the left ventricle, which in turn pumps it to the upper body.
4.With the first breaths, resistance to pulmonary blood flow falls and the volume of blood flowing through the lungs increases six-fold.
5.This results in a rise in the left atrial pressure. Meanwhile, the volume of blood returning to the right atrium falls as the placenta is excluded from the circulation.
6.The change in the pressure difference causes the flap valve of the foramen ovale to be closed.
7.The ductus arteriosus, which connects the pulmonary artery to the aorta in foetal life, will normally close within the first few hours or days.

Question 139

causes dilated cardimyopathy

Answer 139

inherited, secondary to metabolic disease of a result of direct viral infection of the myocardium

Question 140

features cardiomyopathy

Answer 140

an enlarged heart and heart failure who was previously fit and well

Question 141

diagnosis cardio,yopathy

Answer 141

echo

Question 142

mx cardiomyopathy

Answer 142

diuretics and ACEi and carvedilol, a a-adrenoceptor blocking agent. Usually improves spontaneously, but some children may require heart transplants

Question 143

who is at increased risk infective endocarditis

Answer 143

VSD, coarctation of the aorta and PDA or if prosthetic material has been inserted at surgery

Question 144

features IE

Answer 144

Clinical features: Fever, Anaemia and pallor, Splinter haemorrhages in nailbed, Clubbing (late), Necrotic skin lesions, Changing cardiac signs, Splenomegaly, Neurological signs from cerebral infarction, Retinal infarcts, Arthritis/ arthralgia, Haematuria (microscopic

Question 145

diagnosis IE

Answer 145

Blood cultures. Echo. Acute-phase reactants are raised

Question 146

cause IE

Answer 146

= B-haemolytic streptococcus

Question 147

mx IE

Answer 147

Bacterial endocarditis is usually treated with high-dose penicillin in combination with an aminoglycoside (6 weeks IV therapy). Infected prosthetic material may require surgical removal

Question 148

features HF

Answer 148

Symptoms: Breathlessness (particularly on feeding or exertion), Sweating poor feeding, Recurrent chest infections
Signs: Poor weight fain or faltering growth, Tachypnoea, Tachycardia, Heart murmur, gallop rhythm, Enlarged heart, Hepatomegaly, Cool peripheries

Question 149

cause HF

Answer 149

In the first week of life, heart failure usually results from left heart obstruction, e.g. coarctation of the aorta. (Right-to-left shunt)
After the first week, progressive heart failure is most likely due to a left-to-right shunt.

Question 150

eisenmenger syndrome

Answer 150

If a left-to-right shunt is left untreated, these children will develop Eisenmenger syndrome, which is irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow. Now the shunt is right-to-left, and the teenager is blue

Question 151

mx eisenmenger syndrome

Answer 151

heart lung transplant

Question 152

features rheumatic fever

Answer 152

Latent interval of 2-6 weeks following a pharyngeal or skin infection, polyarthritis, mild fever and malaise develop.

Question 153

diagnosing rheumatic fever

Answer 153

two major, or one major and two minor criteria plus supportive evidence of preceding group A streptococcal infection

Question 154

major jones criteria

Answer 154

carditis (endocarditis-significant murmur or valvular dysfunction, myocarditis-may lead to HF, pericarditis-pericardial friction rub, pericardial effusion, tamponade), migratory arthritis (ankles/knees/wrists tender, red, swollen), Sydenham chorea, erythema marginatum, subcutaneous nodules

Question 155

minor jones criteria

Answer 155

fever, polyarthralgia, raised ESR/CRP/leucocytes, prolonged PR on ECG

Question 156

mx rheumatic fever

Answer 156

Acute rheumatic fever = bed rest and anti-inflammatory agents. Corticosteroids may be required if fever and inflammation not resolving. Symptomatic heart failure is treated with diuretics and ACEi. Significant pericardial effusions will require pericardiocentesis
Effective prophylaxis = monthly injections of benzathine penicillin.
Prophylaxis is for 10 years/ until the age of 21 (which is longer).

Question 157

supraventricular tachycardia

Answer 157

Most common child arrhythmia
250-300beats/min

Question 158

presentation supraventicualr tachycardiq

Answer 158

Poor cardiac output, pulmonary oedema, hydrops fetalis/intrauterine death

Question 159

ECG supraventrivcualr tachcyardi

Answer 159

re-entry tachcyardia
narrow complex
250-300bpm

Question 160

mx supraventricualr tachcyardia

Answer 160

Restore sinus rhythm 🡪 IV adenosine
Maintenance therapy 🡪 flecainide/ sotalol

Question 161

congenital heart block

Answer 161

Related to maternal anti-ro/anti-La antibodies
Prevents normal development of the electrical conduction system in the developing heart 🡪 Atrophy and fibrosis of the atrioventricular node

Question 162

presentation congenital heart block

Answer 162

Presyncope / syncope
Death in utero/heart failure in neonates

Question 163

ECG congenital heart block

Answer 163

P and QRS are dissociated

Question 164

mx congenital heart block

Answer 164

an endocardial pacemaker

Question 165

long QT syndrome

Answer 165

Autosomal dominant
Assess if family hx of sudden death / syncope

Question 166

presentation lock QT syndrome

Answer 166

Sudden LOC during exercise/stress

Question 167

mx long QT syndrome

Answer 167

Bblockers, pacemaker

Question 168

ductus venosus role and remnant

Answer 168

allows blood from the placenta to bypass the highly demanding, but relatively inactive liver -> ligamentum venosum

Question 169

ductus arteriosus role and remnant

Answer 169

allowing blood to pass straight from the right ventricle into the aorta and bypass the inactive lungs ->ligamentum arteriosum

Question 170

forament ovale role and remnant

Answer 170

creates a shunt between the right atrium and the left atrium so oxygenated blood from the placenta can move to the left atrium -> fossa ovalis

Question 171

causes of acute dairrhoea

Answer 171

Infective gastroenteritis, Non-enteric infections e.g. respiratory tract, Food hypersensitivity reactions, NEC, Drugs e.g. antibiotics, Henoch-Schonlein purpura, Intussusception (<4yrs), Haemolytic-uremic syndrome, Pseudomembranous enterocolitis

Question 172

mx acute diarrhoea

Answer 172

Assess hydration and vital signs, pallor (blood loss), abdominal tenderness

Question 173

mx mild/moderate dehydration

Answer 173

Replace fluid and electrolyte losses with oral glucose-electrolyte based rehydration fluid e.g. Dioralyte

Question 174

mx severe dehydration/shock

Answer 174

U&E, creatinine, FBC, blood gas, stool M,C&S/virology, tests for specific disease + IV fluid and electrolyte replacement

Question 175

causes of chronic diarrhoea <2y

Answer 175

Malabsorption e.g. post-infective gastroenteritis syndrome, lactose intolerance, cystic fibrosis, coeliac disease, Food hypersensitivity e.g. to cow’s milk protein, Chronic non-specific diarrhoea (toddler diarrhoea): child is usually thriving, Excessive fluid intake, Protracted infectious gastroenteritis, Immuno-deficiencies, Hirschsprung’s disease, Tumours (secretory diarrhoea), Fabricated induced illne s

Question 176

causes of chronic dirahhoea in older children

Answer 176

IBD, Constipation (spurious diarrhoea), Malabsorption, IBS, Chronic infections, including giardiasis, bacterial overgrowth and pseudomembranous colitis, Laxative abuse (EDs), Excessive fluid intake, Fabricated induced illness

Question 177

ix chronic dirahhoea

Answer 177

Stool microscopy, Blood: U&E, FBC, increased CRP/ESR. Radiology: AXR, US, barium meal and follow through

Question 178

causes acute vomiting

Answer 178

infection, GI obstruction (congenital or acquired e.g. pyloric stenosis), adverse food reaction, poisoning, raised ICP, DKA

Question 179

causes chronic vomiting

Answer 179

eptic ulcers, GORD, chronic infection, gastritis, gastroparesis, food allergy, psychogenic, bulimia, pregnancy. Cyclic: idiopathic, CNS disease, abdominal migraine, endocrine, metabolic, intermittent GI obstruction, fabricated illness

Question 180

consequences of vomiting

Answer 180

Metabolic = Potassium deficiency, Alkalosis, Sodium depletion.
Nutritional .
Mechanical injuries to oesophagus and stomach: Mallory-Weiss, Boerhaave’s syndrome, Tears of the short gastric arteries resulting in shock and hemopritoneum.
Dental: erosions and caries.
Oesophageal stricture, Barrett’s metaplasia, broncho-pulmonary aspiration, FTT, anaemia

Question 181

causes of constipation

Answer 181

= Low fibre diet, Lack of mobility and exercise, Poor colonic motility. Gastrointestinal: Hirschsprung’s disease, Anal disease (infection, stenosis, ectopic, fissure, hypertonic sphincter), Partial intestinal obstruction, Food hypersensitivity, Coeliac disease. Non-gastrointestinal: Hypothyroidism, Hypercalcaemia, Neurological disease e.g. spinal disease, Chronic dehydration e.g. diabetes insipidus, Drugs e.g. opiates and anticholinergics, Sexual abuse

Question 182

ix in constipation

Answer 182

Investigations: If an organic cause is suspected consider: FBC – coeliac antibody screen, thyroid function tests, RAST testing. AXR, rectal biopsy (Hirschsprung’s disease).

Question 183

mx constipation

Answer 183

tx cause. Dietary: increased oral fluid and fibre intake, natural laxatives, e.g. fruit juice. Behavioural measures: toilet footrests, regular 5min toilet time after meals, star charts and rewards for child passing stool. Regular oral faecal softeners e.g. Movicol, lactulose, or sodium docusate, will aid disimpaction. Oral stimulant laxatives, e.g. Senna, sodium picosulphate, may be required

Question 184

abdo pain alarm feature

Answer 184

nvoluntary weight loss, Deceleration of linear growth, GI blood loss (visible or occult), Significant vomiting (incl. bilious, protracted, cyclical), Chronic severe diarrhoea, Persistent right upper or lower quadrant pain, Unexplained fever, Family Hx IBD, Abnormal or unexplained physical findings

Question 185

presentation chronic non specific diarrhoea

Answer 185

toddlers diraahoea
6m-5y
Presents with colicky intestinal pain, increased flatus, abdominal distension, loose stools with undigested food . Child is otherwise well and thriving

Question 186

GORD

Answer 186

reflux is repeated and severe enough to cause harm.
Reflux is very common in infancy and is associated with slow gastric emptying, liquid diet (milk), horizontal posture, and low resting lower oesophageal sphincter (LOS) pressure.

Question 187

GORD presentation

Answer 187

Regurgitation, Non-specific irritability, Rumination , Oesophagitis (heartburn, difficult feeding with crying, painful swallowing, haematemesis), Faltering growth (calorie deficiency due to profuse reflux of ingested calories), Apnoea, Hoarseness, Cough, Stridor, Lower respiratory disease – aspiration, pneumonia, asthma

Question 188

complications GORD

Answer 188

Oesophageal stricture (dysphagia), Barrett’s oesophagus (premalignant intestinal metaplasia), Faltering growth, Anaemia (chronic blood loss), Lower respiratory disease

Question 189

ix GORD

Answer 189

Only appropriate when diagnosis uncertain, and there is poor response to treatment, Upper GI endoscopy, Oesophageal biopsy , 24hr oesophageal pH probe

Question 190

mx GORD

Answer 190

Positioning, thickened milk feeds (infants), small frequent meals, avoid food before sleep, fatty foods, citrus juices, caffeine and carbonated drinks, gastric acid reducing drugs e.g. H2 receptor antagonists (ranitidine) or omeprazole (if oesophagitis) or Gaviscon. Surgery: only indicated if failure of intense medical treatment.

Question 191

causes viral gastroenteritis

Answer 191

Rotavirus (most common), Small round structural virus e.g. winter vomiting disease caused by ‘Norwalk agent’, Enteric adenovirus, Astrovirus, CMV (in immune-comprised patients)

Question 192

presentation viral gastroenteritis

Answer 192

Water diarrhoea (rarely bloody), Vomiting, Cramping abdominal pain, Fever, Dehydration, Electrolyte disturbance, Upper respiratory tract signs common with rotavirus, Vomiting predominates with Norwalk virus

Question 193

mx viral gastroenteritis

Answer 193

Give supportive rehydration orally or with a nasogastric tube, or IV glucose and electrolyte solution

Question 194

causes bacterial gastroenteritis

Answer 194

Salmonella, Campylobacter jejuni, Shigella, Yersinia enterocolitica, Escherichia coli, Clostridium difficile, Bacillus cereus, Vibrio cholerae
Sources of infection include contaminated water, poor food hygiene (meat, fresh produce, chicken, eggs, previously cooked rice), faecal-oral route.

Question 195

presentation bacterial gastroenteritis

Answer 195

As for viral gastroenteritis plus: Malaise, Dysentery (bloody and mucous diarrhoea), Abdominal pain may mimic appendicitis or IBD, Tenesmus (feeling as though you need to pass stools even though your bowel is empty)

Question 196

complications bacterial gastroenteritis

Answer 196

Bacteraemia, Secondary infections (particularly Salmonella, Campylobacter), e.g. pneumonia, osteomyelitis, meningitis, Reiter’s syndrome (Shigella, Campylobacter)

Question 197

ix bacterial gastroenteritis

Answer 197

Stool +/- blood culture (some organisms need specific culture medium), Stool Clostridium difficile toxin, Sigmoidoscopy if IBD or colitis

Question 198

mx bacterial gastroenteritis

Answer 198

Rehydration as for viral gastroenteritis. Consider: Erythromycin if Campylobacte, Oral vancomycin or metronidazole if Clostridium difficile (causes pseudomembranous colitis).

Question 199

dehydration classification

Answer 199

Dehydration in children is classified as mild (<5%), moderate (5-10%) and severe (>10%).

Question 200

presentation dehydration

Answer 200

: Children with mild dehydration appear completely normal on examination. Children with moderate dehydration appear well, but have reduced urine output and dry mucous membranes in the absence of other red flag features. Children with severe dehydration have red flag features including a pale, mottled appearance, cool extremities, tachycardia, tachypnoea and/or hypotension, delayed capillary refill, or a change in consciousness.

Question 201

features crohns disease

Answer 201

Symptoms: Diarrhoea , Abdominal pain, Weight loss/failure to thrive, Systemic symptoms: fatigue, fever, malaise, anorexia
GI Signs: Aphthous ulcers , Abdominal tenderness/mass, Perianal abscess/fistulae/skin tags, Anal strictures, Abdominal distension (UC>CD), RIF mass
Non-GI signs and associations: Fever, Finger clubbing, Anaemia, Skin: erythema nodosum, pyoderma gangrenosum. Joints: arthritis, ankylosing spondylitis. Eyes: iritis, conjunctivitis, episcleritis. Poor growth, Delayed puberty, Sclerosing cholangitis, Renal stones, Nutritional deficiencies e.g. vitamin B12

Question 202

complication crohsn disease

Answer 202

Small bowel obstruction, Toxic dilatation , Abscess formation, Fistulae , Malnutrition

Question 203

ix crohns disease

Answer 203

Colonoscopy and biopsy=Crypt abscesses, granulomas, transmural inflammation. Radiology=Mucosal ‘cobblestone’ appearance, ulceration, dilatation, narrowed segments, fistula, ‘skip lesions

Question 204

mx crohns disease

Answer 204

Mild-moderate = Oral 5-aminosalicylic acid (ASA) dimers, e.g. mesalazine. Moderate to severe = Induce remission with oral prednisolone or IV methylprednisolone, 1-2mg/kg/day until condition improved (<2wks) then wean over 6-8wks.. Antibiotics: e.g. ciprofloxacin or metronidazole, may also be useful. Maintenance treatment: immunomodifiers e.g. azathioprine, ciclosporin, tacrolimus, methotrexate, or infliximab (anti-TNF antibody). Surgery: local surgical resection for severe localised disease e.g. strictures, fistula, may be indicated, but there is a high re-operation rate as inflammation recurrence is universal.

Question 205

features ulcerative colitis

Answer 205

Symptoms: Episodic or chronic diarrhoea, Crampy abdominal discomfort, Bowel frequency relates to severity, Urgency/tenesmus – proctitis, Systemic symptoms: fever, malaise, anorexia, weight loss
Signs: Acute, severe UC – fever, tachycardia, tender distended abdomen. Extra-intestinal signs: Clubbing, Aphthous oral ulcers, Conjunctivitis

Question 206

ix UC

Answer 206

Endoscopy: UC histology – crypt abscesses, mucosal inflammation only, goblet cell depletion. Radiology: Mucosal ulceration, haustration loss, colonic narrowing +/- shortening

Question 207

mx UC

Answer 207

: Mild UC = Mesalamine is the mainstay for remission-induction/maintenance. Moderate UC=Induce remission oral prednisolone. Surgery: total colectomy and ileostomy, and later pouch creation and anal anastomosis, cures UC.

Question 208

pathology coeliac disease

Answer 208

: T-cell responses to gluten in the small bowel causes villous atrophy and malabsorption.

Question 209

resentation coeliac disease

Answer 209

Pallor, Diarrhoea, Pale, bulky floating stools, Anorexia, FTT, Irritability. Later: Apathy, Gross motor developmental delay, Ascites, Peripheral oedema, Anaemia, Delayed puberty, Arthralgia, Hypotonia, muscle wasting, Specific nutritional disorders

Question 210

ix coeliac disease

Answer 210

Low Hb, B12 and ferritin. Antibodies: anti-transglutaminase is single preferred test – check IgA levels, Serum tissue transglutaminase IgA antibody (TTG). Endoscopy small bowel biopsy of the third part of the duodenum shows diffuse, subtotal villous atrophy, increased intraepithelial lymphocytes, and crypt hyperplasia

Question 211

IBS

Answer 211

A mixed group of abdominal symptoms for which no organic cause can be found. Most are probably due to disorders of intestinal motility, enhanced visceral perception or microbial dysbiosis.

Question 212

presentation IBS

Answer 212

: Urgency, Abdominal bloating/distension, Worsening of symptoms after food, Symptoms are chronic >6 months and often exacerbated by stress, menstruation or gastroenteritis.

Question 213

ix IBS

Answer 213

: FBC, CRP, ESR, U&E, Coeliac screen, Faecal calprotectin
Only diagnose IBS if recurrent abdominal pain associated with at least 2 of: Relief by defecation, Altered stool form, Altered bowel frequency (constipation/diarrhoea)

Question 214

mx IBS

Answer 214

Should focus on controlling symptoms, initially using lifestyle/dietary measures, then cognitive therapy or pharmacotherapy if required. Constipation: ensure adequate water and fibre intake and promote physical activity. Diarrhoea: avoid sorbitol sweeteners, alcohol and caffeine. Reduce dietary fibre content, encourage patients to identify their own trigger foods. Colic/bloating: oral antispasmodics e.g. buscopan. Combination probiotics in sufficient doses may help flatulence or bloating. Less alcohol intake. Psychological symptoms/visceral hypersensitivity: emphasize the positive. Sinister pathology has been excluded and symptoms tend to improve over time. Consider CBT and hypnosis.

Question 215

causes poor feeding

Answer 215

Premature birth – most common, Traumatic birth injuries that lead to neurological disorders, such as cerebral palsy, Cleft lip and/or cleft palate, Autism, Neck and head abnormalities, Low birth weight, Respiratory problems, Heart disease.

Question 216

features of poor feeding

Answer 216

Arching the back andbodywhilefeeding, Fussinessor lack of alertness whilefeeding, Refusing to eat and drink food andliquids, Excessively long feeding times, Hoarse, or breathy voice quality, Frequent spitting up and/or vomiting, Recurring pneumonia or respiratory infections, Poorweight gain or growt

Question 217

mx poor feeding

Answer 217

Medications, Food temperature and texture changes, Postural or positioning changes, Mouth exercises to make the mouth muscles stronger

Question 218

failure to threive

Answer 218

Weight is the most sensitive indicator in infants and young children, whilst height is better in the older child.
Definition: Fall across 2 centiles on growth chart

Question 219

causes FTT

Answer 219

not enough food being offered or taken, socioeconomic difficulty, emotional deprivation, unskilled feeding , Decreased appetite e.g. psychological or secondary to chronic illness, Inability to ingest, e.g. GI structural or neurological problems, Excessive food loss, e.g. severe vomiting (GORD, pyloric stenosis, dysmotility), diabetes mellitus (urine), Malabsorption, Increased energy requirements e.g. congenital heart disease, CF, malignancy, sepsis, Impaired utilisation (various syndromes)

Question 220

mx FTT

Answer 220

If it resolves in a few weeks, give positive reinforcement and supervise, subsequent growth as an outpatient. If it persists, admit to hospital and observe under supervised adequate dietary input. Adequate growth suggests a non-organic cause

Question 221

kwashiorkor

Answer 221

Kwashiorkoris a form of severe protein malnutrition characterized by oedema and an enlarged liver with fatty infiltrates. It is caused by sufficient calorie intake, but with insufficient protein consumption, which distinguishes it from marasmus.

Question 222

features kwashiorkor

Answer 222

Growth retardation, Diarrhoea, Apathy, Anorexia, Oedema, Skin/hair depigmentation , Abdominal distension with fatty live

Question 223

ix kwashiorkor

Answer 223

Bloods = hypoalbuminaemia, normo- and microcytic anaemia, low calcium, magnesium, phosphate, and glucose

Question 224

mx kwashiorkor

Answer 224

Correct dehydration and electrolyte imbalance, Treat underlying infection and/or parasitic infections, Treat concurrent/causative disease and nutrient deficiencie

Question 225

marasmus

Answer 225

inadequate energy intake in all forms, including protein.

Question 226

features marasmus

Answer 226

Height is relatively preserved compared to weight, Wasted appearance, Muscle atrophy, Listless, Diarrhoea, Constipation

Question 227

ix marasmus

Answer 227

Low serum albumin, Hb, U&Es, calcium, magnesium, phosphate and glucose, stool MC&S for intestinal ova, cysts and parasites

Question 228

mx marasmus

Answer 228

: Correct dehydration and electrolyte imbalance, Treat underlying infection and/or parasitic infections, Treat concurrent/causative disease and nutrient deficiencies

Question 229

presentation pyloric stenosis

Answer 229

M>F, may be a fhx, projectile non bile stained vomit first 4-6w life, hypochloraemic, hypokalaemic metabolic alkalosis

Question 230

ix pyloric stenosis

Answer 230

Test feed or USS

Question 231

my pyloric stenosis

Answer 231

Correct alkalosis, Ramstedt pyloromyotomy

Question 232

presentation acute appendicitis

Answer 232

Uncommon <3y, Pain is aggravated by movement – right iliac fossa, Child may prefer to lie still with knees flexed, Mild fever, Peritoneal irritation results in involuntary spasm in the muscles of the abdominal wall (guarding).

Question 233

ix acute appendicitis

Answer 233

USS

Question 234

mx acute appendicitis

Answer 234

appendicectomy

Question 235

presentation mesenteric adenitis

Answer 235

central abdo pain and urti

Question 236

mx mesenteric adenitis

Answer 236

conservative

Question 237

presentation intussusception

Answer 237

Telescoping bowel proximal to or at level of ileocaecal valve. 6-9m, colicky pain, diarrhoea, vomiting, sausage shaped mass, red jelly stool, drawing up of legs

Question 238

ix intussusception

Answer 238

USS-target sign, AXR-small bowel obstruction

Question 239

mx intussusception

Answer 239

Reduction and air inflation

Question 240

presentation intestinal malrotation

Answer 240

High caecum at midline. Features in examphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia. May be complicated by volvulus (bile stained vomiting)

Question 241

ix intestinal malrotation

Answer 241

Upper GI contrast study and USS

Question 242

x intestinal malrotation

Answer 242

Laparotomy , if volvulus = ladds procedure

Question 243

presentation hirschsprungs

Answer 243

Absence of ganglion cells from myenteric and submucosal plexuses. Delayed passage of meconium and abdo distension

Question 244

ix hirschsprungs

Answer 244

ful thickness rectal biopsy

Question 245

mx hirschsprungs

Answer 245

Rectal washouts then anorectal pull

Question 246

resentation oesophageal atresia

Answer 246

Associated with trachea-oesophageal fistula and polyhydramnios. Choking, cyanotic spells following aspiration

Question 247

presentation meconium ileus

Answer 247

Delayed passage meconium and abdo distension. Associated with CF

Question 248

ix meconium ileus

Answer 248

AXR = bubbly, will not show fluid level.

Question 249

mx meconium ileus

Answer 249

PR contrast and NG N-acetyl cysteine, Gastrograffin enema, surgery

Question 250

presentation biliary atresia

Answer 250

Jaundice >14d, increased conjugated bilirubin, wt loss, swollen abdo

Question 251

ix biliary atresia

Answer 251

Liver biopsy

Question 252

my biliary atresia

Answer 252

Kasai procedure

Question 253

presentation necrotising enterocolitis

Answer 253

RF=prematurity. Abdo distension and passage of bloody stools

Question 254

ix necrotising enterocolitis

Answer 254

XR=pneumatosis intestinalis and free air

Question 255

mx necrotising enterocolitis

Answer 255

TPN, if perforates laparotomy

Question 256

presentation meckels diverticulum

Answer 256

presence of the embryonic vitelline duct that normally involutes during late foetal development.GI bleeding, obstruction, inflammation, umbilical discharge.

Question 257

ix meckels diverticulum

Answer 257

Technetium 99 scan.

Question 258

mx meckels diverticulum

Answer 258

Laparotomy and resection

Question 259

causes chronic liver failure

Answer 259

Chronic hepatitis (After viral hepatitis B or C), Biliary tree disease e.g. biliary atresia, Toxin-induced e.g. paracetamol, alcohol, Alpha1-antitrypsin deficiency, Autoimmune hepatitis, Wilson’s disease (age >3yrs), Cystic fibrosis, Alagille syndrome or non-syndromic paucity of bile ducts, Tyrosinemia , Primary sclerosing cholangitis, PN-induced, Budd-Chiari syndrome

Question 260

presentation chronic liver failure

Answer 260

Jaundice (not always), GI haemorrhage (portal hypertension and variceal bleeding), Pruritus , FTT, Anaemia, Enlarged hard liver (though liver often small in cirrhosis), Non-tender splenomegaly, Hepatic stigmata e.g. spider naevi, Peripheral oedema and/or ascites, Nutritional disorders e.g. rickets, Developmental delay or deterioration in school performance, Chronic encephalopathy

Question 261

mx chronic liver failure

Answer 261

Treat the underlying cause and give nutritional support - Lower protein, increased energy, higher carbohydrate diet, Vitamin supplementation, particularly fat soluble vitamins A,D,E,K. involve a paediatric dietitian. Drug therapy: Prednisolone +/- azathioprine for autoimmune hepatitis, Interferon-alpha +/- ribavirin for chronic viral hepatitis, Penicillamine for Wilson’s disease, Colestyramine may be useful to control severe pruritus, Vitamin K1 if significant coagulopathy or bleeding. Treat symptoms: Oesophageal varices – endoscopy, Ascites – fluid restriction and spironolactone, Encephalopathy – reduce GI ammonia absorption using oral lactulose, Liver transplant

Question 262

presentation alpha 1 antitrypsin deficiency

Answer 262

Cholestasis in infancy, may progress to liver failure, Cirrhosis can occur in late childhood to adult. Chronic liver disease affects 25% of patients in late adulthood. Pulmonary emphysema is the commonest presentation in adulthood

Question 263

mx alpha 1 antitrypsin deficincy

Answer 263

Treatment: Supportive treatment of liver complications, Liver transplant for end-stage liver failure

Question 264

nheritance wilson

Answer 264

autosomal recessive disorder

Question 265

presentation wilsons

Answer 265

Kayser-Fleischer rings (copper deposition in Descemet’s membrane in the eye) often present and are pathognomonic. May require slit-lamp examination to visualise. Hepatic problems usually present in childhood (hepatitis, cirrhosis, fulminant hepatic failure). Adolescents/ young adults usually present with neurological disease

Question 266

ix wilsons

Answer 266

Serum copper and ceruloplasmin low, 24hr urinary copper excretion >100microgram (normal <40), Molecular genetic testing – Wilson’s gene (ATP7B) mutation

Question 267

mx wilsons

Answer 267

Lifelong chelation therapy with penicillamine (reverses pre-cirrhotic liver disease, but not neurological damage), Liver transplantation if end-stage hepatic failure

Question 268

colic

Answer 268

when an infant who isn’t sick or hungry cries for more than 3 hours a day, more than 3 days a week, for more than 3 weeks.

Question 269

presentation colic

Answer 269

likely to start around 2 weeks of age if your infant is full-term, or later if they were born prematurely.
Features: It’s hard to soothe or settle your baby, They clench their fists, They go red in the face, They bring their knees up to their tummy or arch their back, Their tummy rumbles or they’re very windy

Question 270

inguinal hernia features

Answer 270

More common in boys, More common on the right side, usually asymptomatic, a reducible swelling in groin

Question 271

mx inguinal hernia

Answer 271

Surgical herniotomy. Infants should be repaired within a few weeks of diagnosis because the risk of incarceration is high.
incarcerated hernia: results in an intestinal obstruction

Question 272

mx umbilical hernia

Answer 272

Most will close spontaneously during the first few years of life, regardless of size
If the hernia fails to close surgical repair can be performed at around 5yrs of age

Question 273

presentation congenital diaphragmatic hernia

Answer 273

Respiratory distress – tachypnoea, cyanosis, grunting. Scaphoid abdomen (because bowel in chest), Apparent dextrocardia (mediastinum displaced into right thorax, Bowel sounds may be audible in chest (uncommon)

Question 274

mx congenital diaphragmatic hernia

Answer 274

Initial management consists of sedation, paralysis, endotracheal intubation, and mechanical ventilation with 100% O2, repair of the diaphragmatic defect is undertaken after a few days either by primary suture or insertion of a prosthetic patch

Question 275

hiatus hernia

Answer 275

herniation of the stomach into the chest through the oesophageal hiatus in the diaphragm. The lower oesophageal sphincter also moves and becomes incompetent
Two types of hiatus hernia are recognised: Sliding (common), Rolling or paraesophageal (rare)

Question 276

mx hiatus hernia

Answer 276

Diagnosis is made radiologically by barium meal. Surgery is reserved for children who fail to respond to medication, complicated reflux (e.g. peptic strictures), and paraesophageal hernias

Question 277

presentration cows milk allergy

Answer 277

Symptoms depend on where the allergic inflammation is: Upper GI tract – vomiting, feeding aversion, pain. Small intestine – diarrhoea, abdominal pain, protein-losing enteropathy, FTT. Large intestine – diarrhoea, acute colitis with blood and mucus in stools, rarely chronic constipation

Question 278

mx cows milk allergy

Answer 278

, first treat by limiting cow’s milk protein intake (and commonly soy protein). In exclusively breast-fed infants, this is achieved by a maternal exclusion diet to these proteins. In formula fed infants feed with a hydrolysed formula (short peptides)

Question 279

presentation UTI

Answer 279

: Vomiting, poor feeding, with or without abdominal pain or tenderness, Lethargy or irritability, Urinary frequency or dysuria, Haematuria

Question 280

ix UTI

Answer 280

> 10,5 organisms/mL in pure growth from a carefully collected urine sample (midstream urine, clean catch urine, or bag urine).
Investigations: Dipstick test in the urine. ‘Leucocytes’ and ‘nitrites’ strongly suggests UTI. Urine should be sent for microscopy, culture, and sensitivity,

Question 281

mx UTI

Answer 281

Antibiotics should be started after urine collection e.g. Trimethoprim, Co-amoxiclav. Infants less than 3 months old should be referred immediately to a paediatrician. Children aged more than 3 months old with an upper UTI should be considered for admission to hospital. If not admitted oral antibiotics such as cephalosporin or co-amoxiclav should be given for 7-10 days. Children aged more than 3 months old with a lower UTI should be treated with oral antibiotics for 3 days according to local guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin. Parents should be asked to bring the children back if they remain unwell after 24-48 hours

Question 282

primary nocturnal enuresis

Answer 282

Majority of cases have no underlying organic cause and it is thought to be due to delayed maturation of bladder control mechanisms.

Question 283

secondary nocturnal enuresis

Answer 283

UTI, spina bifida, diabetes mellitus, diabetes insipidus, Behavioural problems, Abuse

Question 284

nocnturnal enuresis

Answer 284

Encourage regular drinks (water), but restrict in last hour before bed, Give drinking/ voiding chart, Enuresis alarm, >7 years: desmopressin can be considered

Question 285

pre renal causes AKI

Answer 285

Hypovolaemia e.g. gastroenteritis, haemorrhage, DKA, nephrotic syndrome, Peripheral vasodilatation e.g. sepsis, Impaired cardiac output e.g. congestive cardiac failure, Drugs e.g. ACE inhibitors

Question 286

renal causes AKI

Answer 286

Acute tubular necrosis, Interstitial nephritis, Glomerulonephritis, Haemolytic-uraemic syndrome, Cortical necrosis, Bilateral pyelonephritis, Nephrotoxic drugs e.g. aminoglycoside, IV contrast, NSAIDs, Myoglobinuria, haemoglobinuria, Tumour lysis syndrome, Renal artery/ vein thrombosis

Question 287

post renal causes AKI

Answer 287

Obstruction, Post-urethral valves, Neurogenic bladder , Calculi, Tumours (rhabdomyosarcoma in infancy)

Question 288

mx AKI

Answer 288

Treat electrolyte imbalances and shock. Fluids management: pre-renal – fluid bolus and furosemide. Otherwise restrict insensible losses + urine output.
Indications for dialysis: Severe hyperkalaemia, Symptomatic uraemia with vomiting/encephalopathy , Rapidly rising urea and creatinine, Symptomatic fluid overload, especially cardiac failure or pericardial effusion, Uncontrollable hypertension, Symptomatic electrolyte problems or acidosis, Encephalopathy or seizures, Prolonged oliguria: conservative regimen controls ARF, but causes nutritional failure, Removal exogenous toxins or metabolites (inborn error)

Question 289

presentation CKD

Answer 289

Failure to thrive, Polyuria and polydipsia, Lethargy, lack of energy, poor school concentration, Other abnormalities such as ricket

Question 290

auses CKD

Answer 290

Congenital =Renal dysplasia, Obstructive uropathies, Vesicoureteric reflux nephropathy. Hereditary =PKD, Hereditary nephritis, Cystinosis, Oxalosis . Glomerulopathies= Focal segmental glomerulosclerosis. Multisystem disorders=SLE, HSP, Haemolytic-uraemic syndrome. Others=Wilms’ tumour, Renal vascular disease, Unknown

Question 291

hypospadias

Answer 291

A birth defect in which the opening of the urethra is on the underside of the penis instead of at the tip.

Question 292

consequences hypospadias

Answer 292

difficulty urinating while standing and a cosmetic appearance Sexual function is not affected unless chordee, which may cause painful erections, is present

Question 293

mx hypospadias

Answer 293

do not circumcise, surgery

Question 294

vesicoureteral reflux

Answer 294

retrograde flow of urine from the bladder into the upper urinary tract.
Grade of VUR: I-V

Question 295

diagnosis vesicoureteral reflux

Answer 295

Micturating cystourethrogram – requires catheter, Indirect cystogram

Question 296

mx vesicoureteral reflux

Answer 296

Prophylactic antibiotics against UTI, surgery

Question 297

features haemolytic uraemic syndrome

Answer 297

A group of blood disorders characterized by low red blood cells, acute kidney failure, and low platelets.
Initial symptoms typically include bloody diarrhoea, fever, vomiting, and weakness. Kidney problems and low platelets then occur as the diarrhoea is improving.
Microangiopathic haemolytic anaemia, Thrombocytopenia, Acute kidney failure

Question 298

cause HUS

Answer 298

e.coli

Question 299

phimosis

Answer 299

tigh foreskin

Question 300

nephrotic syndrome

Answer 300

Defined as a combination of: Heavy proteinuria (urinary protein to creatinine ratio >200mg/mmol), Hypoalbuminemia (albumin <25g/L), Oedema, Hyperlipidaemia

Question 301

causes nephrotic syndrome

Answer 301

Primary =Congenital,Infantile. Secondary =Minimal change disease (MCD): commonest, Focal segmental glomerulosclerosis, Membranoproliferative glomerulonephritis, Membranous glomerulonephritis

Question 302

mx nephrotc syndrome

Answer 302

: fluid restriction and prevention of hypovolaemia , Trial of oral steroid therapy to induce remission is also started, Prophylaxis against bacterial infection (particularly pneumococcal)

Question 303

complications nephrotic syndrome

Answer 303

Predisposition to infection is due to decreased IgG levels, Thrombosis, Hypovolaemia

Question 304

minimal change disease

Answer 304

most common cause of nephrotic syndrome in children (~70%) and is characterised by minimal histological changes in the kidney structure

Question 305

presentation minnimal change disease

Answer 305

facial swelling and are most commonly 1-8 years old. May be asymptomatic

Question 306

mx minimal change disease

Answer 306

Fluid restriction and reduced salt intake, Corticosteroid therapy: prednisolone, Human albumin and furosemide

Question 307

complications minimal change diseas

Answer 307

Spontaneous peritonitis, Thrombosis, Recurrent minimal change disease, Hypertension

Question 308

nephritic syndrome

Answer 308

Haematuria, Oedema (to a lesser extent compared to nephrotic syndrome), Reduced urine output, Uraemic symptoms (e.g. reduced appetite, fatigue, pruritus, nausea), HTN, mild proteinuria

Question 309

causes neohritic syndrome

Answer 309

Post-infectious = Bacterial: streptococcal commonest, Staphylococcus aureus, Mycoplasma pneumoniae, Salmonella, Virus: herpes viruses (EBV, varicella, CMV), Fungi: candida, aspergillus,Parasites: toxoplasma, malaria, schistosomiasis . Others (less common) = MPGN (membranoproliferative glomerulonephritis), IgA nephropathy, SLE, Subacute bacterial endocarditis, Shunt nephritis

Question 310

mx nephritic syndrome

Answer 310

fluid balance and restrict salt, mx HTN with alpha blocker or CCB

Question 311

features post strep glomerulonephritis

Answer 311

Usually presents 1-2weeks after a URTI and sore throat, treat with antibiotics (penicillin)

Question 312

features HSP

Answer 312

Small vessel vasculitis associated with IgA immune complexes. A triad of arthritis, colicky abdominal pain, and palpable, purpuric rash over buttocks and extensor surfaces of arms and legs.Characteristically affects prepubertal boys. Usually seen in children following an infection. Manage with NSAIDs and corticosteroids

Question 313

features alport syndrome

Answer 313

Sensorineural deafness with progressive nephritis.

Question 314

features SLE

Answer 314

Complex, multisystem AI disorder affecting adolescents. Commoner and more severe in Afro-Caribbean, Hispanic, and Far Eastern girls. The revised ARA criteria for classification of SLE –diagnosed if 4/11 present of: Malar rash, Discoid rash, Photosensitivity, Mouth ulcers, Arthritis (non-erosive), Serositis: pleurisy or pericarditis, Renal disease: persistent proteinuria >0.5g/24hr or cellular casts, Neurological disorder: psychosis or seizures in absence of known precipitants, Haematological abnormality: haemolytic anaemia or leucopoenia, Immunological: raised anti DNA binding antibody, +ve antiphospholipid antibodies, Antinuclear. Management in specialist clinic: avoids sun exposure, ACEi, NSAIDs, hydroxychloroquine, prednisolone

Question 315

features anaohylaxis

Answer 315

Skin: urticaria and angioedema, Respiratory: acute airway obstruction with laryngeal oedema and bronchospasm, swollen tongue/lips, sneezing, wheeze, Gastrointestinal: severe abdominal cramping and diarrhoea, Systemic: tachycardia, hypotension and shock

Question 316

mx anaphylaxis

Answer 316

remove trigger, call for help, A-E assessment, IM adrenaline (150micrograms <6, 300 6-12, 500>12. Also chlorphenamine and hydrocortisone in hospital

Question 317

causes necrotising fascitis

Answer 317

: type 1 is caused by mixed anaerobes and aerobes(often occurs post-surgery in diabetics). type 2 is caused byStreptococcus pyogenes

Question 318

features necrotising fascitis

Answer 318

Acute onset, Pain, swelling, erythema at the affected site-often presents asrapidly worsening cellulitis with pain out of keeping with physical features, extremely tender over infected tissue with hypoaesthesia to light touch, skin necrosis and crepitus/gas gangrene are late signs. Fever and tachycardia may be absent or occur late in the presentation

Question 319

mx necrotising fascitis

Answer 319

Urgent surgical referral debridement, Intravenous antibiotics

Question 320

presentation allergic rhinits

Answer 320

nasal congestion, itching, sneezing, and discharge.

Question 321

ix allergic rhinitis

Answer 321

Skin tests for specific antigens, Specific serum IgE measurements

Question 322

mx allergic rhinitis

Answer 322

Allergen avoidance, Symptom relief=Antihistamines, Montelukast ,Intranasal steroid

Question 323

cause stephens johnson syndrome

Answer 323

usually drug induced with viral infection rarely implicated

Question 324

resentation stephen jonson syndrome

Answer 324

Widespread blisters/ bullae over erythematous, purple macular, or haemorrhage skin, Mucous membranes often affected with haemorrhagic crusting, Rubbing may cause skin separation at epidermodermal junction (= positive Nikolsky sign), Also possible fever, arthralgia, myalgia, prostration, renal failure, pneumonitis, conjunctivitis, corneal ulceration, blindness

Question 325

mx stephen johnson syndrome

Answer 325

Supportive, as for severe burns (e.g. hydration, airway protection), Frequent emollient ointment, Specialist eye care , Systemic corticosteroids or immunoglobulin used in first 2-3 days may be helpful if life-threatening.

Question 326

pathophysiology urticaria

Answer 326

: Adverse stimulus -> mast cell degranulation -> histamine release -> localised vasodilatation and increased capillary permeability.

Question 327

causes urticaria

Answer 327

: Usually idiopathic or triggered by recent viral infection. Often causes include: Allergens (e.g. drugs, foods, inhalants, insect bites), Trauma (physical urticarias) e.g. dermographism due to light skin trauma (commonest), pressure, cold, heat, sunlight.
Chronic urticaria (defined as acute urticaria not resolving after 2 mths) is idiopathic in >90%, but may be caused by: Chronic bacterial, fungal (e.g. oral Candida), or parasitic infection

Question 328

presnetaion urticaria

Answer 328

Rapidly developing erythematous eruption with raised central white wheals and occasionally local purpura, Lesions last 4-24hr

Question 329

mx urticaria

Answer 329

Oral antihistamines, Oral prednisolone, short course if severe

Question 330

presentation angiodema

Answer 330

Variant of urticaria with significant swelling of subcutaneous tissues, often involves lips, eyelids, genitals, tongue, or larynx. If severe, may cause acute upper or lower respiratory tract obstruction and may be life-threatening

Question 331

mx angiodema

Answer 331

Give facial oxygen , IM 0.1mL/kg adrenaline 1:10,000, IM/IV hydrocortisone 12-hourly, Nebulised salbutamol

Question 332

presentation staph scalded skin syndrome

Answer 332

Extensive tender erythema with flaccid superficial blisters/bullae (scalded appearance), Erosions and +ve Nikolsky sign, Crusting around eyes and mouth, fever

Question 333

mx staph scalded skin syndrome

Answer 333

: Supportive treatment and analgesia, IV anti-staphylococcal antibiotics, Gentle skin care, emollient ointments

Question 334

presentation infantile seborrheic eczema

Answer 334

Usually appears after a few weeks. Erythema and scaling affects face, neck, behind ears, axillae, scalp, upper trunk, napkin area, and flexures

Question 335

mx infantile seborrheic eczema

Answer 335

Avoid detergents e.g. soap, Use topical emollients, Mild topical steroid/antifungal preparation e.g. 1% hydrocortisone cream

Question 336

cause atopic eczema

Answer 336

: Genetic susceptibility , Impaired epidermal barrier function, Immune dysregulation, Allergen (food and airborne) sensitisation and infection play a lesser role

Question 337

presentation atopic eczema

Answer 337

Acute eczema may be erythematous and weeping, Chronic eczema may be lichenified and dry, These are often 2° changes of excoriation, post-inflammatory hypo/hyperpigmentation and infection, Infant eczema often affects cheeks, elbows, and knees with crawling, Childhood eczema is often flexural, also affects the wrists and ankles, Adolescent and adult eczema is also flexural, but may also affect the head and neck, nipples, palms, and soles

Question 338

mx atopic eczema

Answer 338

General measures: soap avoidance, e.g. soap free bath oil or wash. Limit showers/ baths to 5-10 min in lukewarm water. Moisturise immediately after showering. Wear loose fitting cotton undergarments. Avoid irritants. Mild eczema= A mild potency topical corticosteroid ointment e.g. 1% hydrocortisone, is appropriate daily for anywhere on the body. Mild to moderate eczema=A moderate potency topical corticosteroid ointment e.g. clobetasone butyrate 0.05% is appropriate and safe for daily use on the face and body, but not the groin. Moderate to severe eczema=A potent topical corticosteroid ointment e.g. mometasone furoate, is appropriate daily for the body, but not the face or groin.

Question 339

omplications atopic eczema

Answer 339

Sleep disturbance, Emotional upset, Family dysfunction, Eczema herpeticum (HSV), Staphylococcus aureus infection, Growth delay , Atopic cataracts

Question 340

cause reyers syndrome

Answer 340

aspirin

Question 341

sentation of reyes syndrome

Answer 341

: Abnormal liver function tests, vomiting and encephalopathy (slurred speech, lethargy, coma and potentially death).

Question 342

cavernous haemangioma

Answer 342

Wouldn’t want these near the airway or covering the eye or places where they may get knocked easily and bleed.
Contains lots of blood vessels that can bleed easily
90% of them go away by 9 years, leave a tissue-paper scar (thin layer of skin)
temmporary

Question 343

capillary haemangioma

Answer 343

permanent
Most are just cosmetic nuisance but be cautious of Sturge Weber syndrome
A condition that affects the development of certain blood vessels, causing abnormalities in the eyes, skin and brain.

Question 344

a naevi

Answer 344

mole

Question 345

mongolian blue spots

Answer 345

non-caucasian ancestory

Question 346

cafe au lait patch

Answer 346

more than 5=neurofibromatosis II
A genetic condition characterised primarily by changes in skin colour and the growth of benign tumours along the nerves of the skin, brain and other parts of the body

Question 347

milia

Answer 347

Sebaceous plugs
Nothing to worry about

Question 348

causes od delayed walking

Answer 348

Cerebral palsy, Duchenne muscular dystrophy (DMD), Global neurodevelopmental delay as part of a syndrome or other unidentified cause

Question 349

causes od delayed speech

Answer 349

Familial: a family history of language delay where parents have been late in developing language skills or have had speech therapy
Hearing impairment: chronic otitis media (glue ear) is a common cause for delayed or poor clarity or speech in the pre-school age
Environmental: poor social interaction/deprivation
Neuropsychological: Global developmental delay, ASD

Question 350

causes of global neurodevelopmental delay

Answer 350

Genetic: Chromosomal disorders e.g. Down syndrome, fragile X, Duchenne muscular dystrophy, Metabolic syndromes e.g. phenylketonuria
Congenital brain anomalies e.g. hydrocephalus or microcephaly
Prenatal insult: Teratogens e.g. alcohol and drugs, Congenital infections e.g. rubella, CMV, or toxoplasmosis, Hypothyroidism
Perinatal insult: Complication of extreme prematurity e.g. intraventricular haemorrhage; periventricular leukomalacia, Birth asphyxia, Metabolic disorder e.g. hypoglycaemia or hyperbilirubinemia
Postnatal events: Brain injury: trauma; anoxia e.g. suffocation or drowning, CNS infection: e.g. encephalitis/meningitis, Metabolic: e.g. hypoglycaemia

Question 351

dyspraxia

Answer 351

A disorder of motor planning and/or execution with no significant findings on standard neurological examinations.. A disorder of the higher cortical process and there may be associated problems of perception, use of language and putting thoughts together.
Features include problems with: Handwriting, which is typically awkward, messy, slow, irregular and poorly spaced, Dressing (buttons, laces, clothes), Cutting up food, Poorly established laterality , Copying and drawing, Messy eating from difficulty in coordinating biting, chewing and swallowing (oromotor dyspraxia). Dribbling is common.
Verbal dyspraxia is where there are more specific difficulties related to speech production in the absence of muscle or nerve damage

Question 352

dyslexia

Answer 352

A disorder of reading skills disproportionate to the child’s IQ.
The term is often used when the child’s reading age is more than 2 years behind his/her chronological age

Question 353

newborn gross motor

Answer 353

Flexed arms and legs
Equal movements

Question 354

3m gross motor

Answer 354

Lifts head on tummy

Question 355

6m gross motor

Answer 355

Chest up with arm support
Rolls
Sit unsupported

Question 356

9m gross motor

Answer 356

Pulls to stand

Question 357

1yr+ gross motor

Answer 357

1 year - walking
2 years - walks up stairs
3 years - jumps
4 years - hops
5 years - rides a bike

Question 358

4m fine motor and vision

Answer 358

Grasp an object
Uses both hands

Question 359

8m fine motor and f=vision

Answer 359

Takes a cube in each hand

Question 360

1yr+ fine motor and vision

Answer 360

12 months
Scribbles with a crayon

18 months
Builds a tower of 2 cubes

3 years
Tower of 8 cubes

Question 361

speech and language 3m

Answer 361

Laughs and squeals

Question 362

peech and langage 9m

Answer 362

dada, mama

Question 363

speech and langage 1yr

Answer 363

1 word

Question 364

speech and language 2y

Answer 364

2 words sentences,
Names body parts but not good at localising pain

Question 365

speech and langiage 3y+

Answer 365

3 years Speech mainly understandable
4 years Knows colours, can count 5 objects
5 years Knows meaning of words e.g. what is a lake?

Question 366

social 6w

Answer 366

Smiles spontaneously

Question 367

ocial 6m

Answer 367

finger feeds

Question 368

social 9m

Answer 368

waves bye

Question 369

social 12m

Answer 369

uses spoon/fork

Question 370

social 2y+

Answer 370

2 years: Takes some clothes off, Feed a doll, Play alongside but not with others
3 years: Play with others, name a friend, Put on a t-shirt
4 years: Dress no help, Play a board game

Question 371

concerninc milestones

Answer 371

Gross motor
Not sitting by 1 year
Not walking at 18 months

Fine motor
Hand preference before 18 months - one side developing really early can indicate a problem with the other side (cerebral palsy)

Speech and language
Not smiling by 3 months
No clear words by 18 months

Social development
No response to carers interactions by 8 weeks
Not interested in playing with peers by 3 years - autism?

Question 372

development red flags

Answer 372

Regression
Poor health/growth
Significant family history
Findings on examination e.g. microcephaly, dysmorphic features
Safeguarding indicators

Question 373

presentation febrile convulsions

Answer 373

viral infection, brief generalised tonic-clonic seizures.

Question 374

mx febrile convulsion

Answer 374

reduced temp- paracetamol

Question 375

cause reflex anoxic seizures

Answer 375

a precipitating trigger causes over-stimulation of the vagus nerve, which reduces heart rate and cardiac output. This reduces blood flow to the brain

Question 376

resentation reflex anoxic seizures

Answer 376

Pale, falls to floor, rapid recovery

Question 377

resentation breath holding spells

Answer 377

hold their breath during periods of crying to the point that they faint, turning blue and jerking of the limbs, recovers quickly

Question 378

presentation west yndrome

Answer 378

4-8m, Characteristic ‘salaam’ attacks: flexion of the head, trunk and arms followed by extension of the arms, lasts only 1-2 seconds but may be repeated up to 50 times. Progressive mental handicap. EEG shows hypsarrhythmia in two-thirds of infants
CT demonstrates diffuse or localised brain disease sclerosis)

Question 379

mx west syndrome

Answer 379

poor prognosis, Vigabatrin is now considered first-line therapy
ACTH is also used

Question 380

presntation absence seizures

Answer 380

4-8y, Duration few-30 secs; no warning, quick recovery; often many per day. EEG: 3Hz generalized, symmetrical

Question 381

mx absenze seizures

Answer 381

Sodium valproate, ethosuximide

Question 382

presentation lennox gastaut syndrome`

Answer 382

1-6yAtypical absences, falls, jerks
90% moderate-severe mental handicap. EEG: slow spike

Question 383

lennox gastaut syndrome

Answer 383

?ketotgenic diet

Question 384

presentation benign rolandic epilepsy

Answer 384

Paraesthesia (e.g. unilateral face), usually on waking up

Question 385

presntation juvenile myoclonic epilspsy

Answer 385

teens, Infrequent generalized seizures, often inmorning//following sleep deprivation, Daytime absences
Sudden, shock like myoclonic seizure(these may develop before seizures

Question 386

mx juveline myoclonic epilsosy

Answer 386

sodium valproate

Question 387

presentation focal seizures

Answer 387

Frontal=jacksonian march. Temporal=déjà vu, lip smacking. Occipital=hallucinations. Parietal=odd sensations

Question 388

mx focal seizures

Answer 388

valproate, levetiracetam

Question 389

cause impetigo

Answer 389

Staphylococcal aureus or streptococcal skin infection

Question 390

presentation impetigo

Answer 390

: erythematous macules (may progress to be vesicular/bullous) on face, neck or hands

Question 391

mx impetigo

Answer 391

topical (fusidic acid, mupirocin) or systemic (flucloxacillin or clarithromycin)

Question 392

kawasaki disease

Answer 392

an acute systemic vasculitis that affects young children

Question 393

cause kawasaki disease

Answer 393

autoimmune-mediated (medium-sized blood vessel vasculitis

Question 394

presentation kawasaki disease

Answer 394

fever > 5 days, conjunctivitis, polymorphous exanthem, fissuring of lips, strawberry tongue (Figure 4), diffuse erythema of oral and pharyngeal mucosa, periungual desquamation of fingers and toes, erythema of palms and soles
Other features: arthralgia, septic meningitis, coronary artery aneurysm, vasculitis and other cardiac conditions (congestive heart failure, myocarditis, arrhythmias, mitral insufficiency, acute MI)

Question 395

ix kawasaki disease

Answer 395

echocardiography (needs follow up 6 weeks later), inflammatory markers (ESR and CRP), alpha-1 antitrypsin

Question 396

mx kawasaki disease

Answer 396

high dose intravenous immunoglobulin, aspirin

Question 397

presentation erythema nodosum

Answer 397

red or violet subcutaneous nodules located pretibially

Question 398

cause erythema nodosum

Answer 398

streptococcal pharyngitis, idiopathic, sarcoidosis, primary tuberculosis, inflammatory bowel disease, drug reactions

Question 399

mx erythema nodosum

Answer 399

usually self-limiting or resolves with the treatment of the underlying disorder

Question 400

cause measles

Answer 400

caused by a morbillivirus of the paramyxovirus family.

Question 401

presentation measles

Answer 401

Appearance: maculopapular rash lasts 6-8 days
Associated symptoms: fever, coryza, cough, non-purulent conjunctivitis, Koplik spots
Epidemiology: young children with a seasonal peak in late winter/spring

Question 402

mx measles

Answer 402

supportive, can give antibiotics to prevent secondary infection

Question 403

cause eczema herpeticum

Answer 403

type I HSV co-infection with active atopic eczema

Question 404

presentation eczema herpeticum

Answer 404

Primary infection: in pre-school children, presenting with a sore throat, pyrexia, stomatitis, vesicles or ulceration in the oral cavity and face
Secondary infection: a cluster of itchy and painful blisters on the face and neck. New blisters have umbilication, old blisters crust and form sores

Question 405

mx eczema herpeticum

Answer 405

oral acyclovir, systemic antibiotics for secondary bacterial infection

Question 406

erythema multiforme

Answer 406

type IV hypersensitivity reaction that presents with a skin rash.

Question 407

cause erythema multiforme

Answer 407

: HSV (90%), mycoplasma pneumonia, medications, autoimmune disease, sarcoidosis

Question 408

resentation erythema multiforme

Answer 408

target-like lesions on the skin. Progresses to erosions of bullae which can involve oral, genital or mucosal areas.

Question 409

mx erythema multiforme

Answer 409

usually self-limiting, aciclovir is used to treat HSV infections. Oral antihistamines and corticosteroids can be used to reduce pruritus.

Question 410

cause glandular fever

Answer 410

Epstein-Barr virus

Question 411

presntation glandular fever

Answer 411

fever, fatigue, sore throat, lymphadenopathy
A maculopapular rash can occur due to being treated with penicillin whilst infected with EBV

Question 412

cause hand, foot and mouth disease

Answer 412

coxsackievirus A16 and enterovirus A71

Question 413

rsentation hand, foot and mouth disease

Answer 413

oral vesicles which rupture to form ulcers on tongue and buccal mucosa (enanthem). Macular, maculopapular or vesicular exanthema on hands, feet, buttocks, legs, arms.

Question 414

mx hand foot and mouth disease

Answer 414

supportive, but children with complications may require hospitalization

Question 415

cause fifth disease/erythrma infectiosum

Answer 415

parvovirus B19

Question 416

presentation fitfh disease

Answer 416

begins with fever, coryza, headache, nausea and vomiting
Appearance: malar rash with circumoral pallor (slapped cheek rash), then a lace-like rash on trunk and extremities follows

Question 417

cause chicken pox

Answer 417

caused by the varicella-zoster virus (VZV).

Question 418

presentation chicken pox

Answer 418

Appearance: starts on head and trunk, then spreads throughout the body. Red macules -> papules -> pustule-> crusting
Epidemiology: between 1 and 6 years, seasonal peaks in winter and spring
Associated symptoms: headache, anorexia, upper respiratory tract infection, fever, itching

Question 419

mx chickenpox

Answer 419

antihistamines, paracetamol, acyclovir, VZIG for prophylaxis for contact at-risk individual

Question 420

what is nappy rash

Answer 420

irritant contact dermatitis that occurs in the nappy area
Secondary infection with Candida albicans or bacteria (Staphylococcal aureus or streptococcus) can occur.

Question 421

resentation nappy rash

Answer 421

beefy red plaques, satellite papules, superficial pustules

Question 422

mx nappy rash

Answer 422

frequent application of emollients, topical antifungal agent (e.g. nystatin, clotrimazole or ketoconazole)

Question 423

ause scabies

Answer 423

an infestation of the skin by mite Sarcoptes scabiei resulting in a pruritic eruption

Question 424

presentation scabies

Answer 424

small, erythematous papule with haemorrhagic crusts on fingers, elbows, axillary folds, thighs, genitalia, feet

Question 425

mx scabies

Answer 425

hygiene advice, topical permethrin, oral ivermectin

Question 426

cause tinea corporis

Answer 426

Trichophyton tubrum, Microsporum canis, Epidermophyton

Question 427

resentation tinea corporis

Answer 427

pruritic, circular, erythematous scaly patch spreading centrifugally. Central clearing is seen.

Question 428

mx tinea corporis

Answer 428

daily application of topical antifungals. Systemic therapy indicated in patients with failed topical therapy (terbinafine, fluconazole or itraconazole).

Question 429

presntation tinea capitis

Answer 429

well-demarcated scaly lesion, can be “grey patch”, “black dot” and favus

Question 430

mx tinea capitis

Answer 430

: systemic treatment with oral griseofulvin/terbinafine

Question 431

cause molluscum contagiosum

Answer 431

poxvirus

Question 432

presentation molluscum contagiosum

Answer 432

flesh-coloured, dome-shaped papules on the skin
Associated symptoms: usually painless and sometimes pruritic

Question 433

mxmolluscum contagiosum

Answer 433

self-resolving after approximately 18 months

Question 434

cause scarlet fever

Answer 434

strep pyogenes

Question 435

presentation scarlet ever

Answer 435

Develops within 48h of acute tonsillopharyngitis, strep throat

Rash: scarlet-coloured, pruritic, skin like sandpaper, begins on neck and spreads, pastias lines, after a week the rash fades and desquamates

Question 436

mx scarlet fever

Answer 436

10 oral penicllin V

Question 437

presentation rubella

Answer 437

flu like, post auricular and suboccipital lymphadenopathy, forchheimer spots, maculopapular rash starting behind ears

Question 438

presentation polio

Answer 438

Presentation: asymptomatic, flu like illness, less than 1% develop paralytic polio

Question 439

presnetation diphtheriaa

Answer 439

thick grey-white coating at the back of your throat, temp, nausea, sore throat, headache,

Question 440

mx diphtheria

Answer 440

Antitoxin, Antibiotics – erythromycin or IM penicillin to eradicate

Question 441

presnetation TB

Answer 441

Starts with: Febrile illness, Erythema nodosum ,Phlyctenular conjunctivitis
6-9months: In most cases progressive healing of primary complex, effusion, cavitation, military spread

Question 442

ix TB

Answer 442

Mantoux test, sputum sample=acid fast bacili

Question 443

mx Tb

Answer 443

Isoniazid – SE: burning sensation in feet, Rifampicin – SE: orange/ red urine, Pyrazinamide – SE: joint pains, Ethambutol – SE: visual impairment

Question 444

AIDS defnining illnesses

Answer 444

Lymphocytic interstitial pneumonitis, Pneumocystis carinii (PCP) infection, and Candida oesophagitis are ‘AIDS defining’ in an HIV-positive child, they signify progression to the AIDS phase.

Question 445

diagnosing HIV

Answer 445

Specific antibody response (anti-HIV antibodies): infants infected perinatally have an immune response by 4-6months of age. However, an uninfected infant of a HIV-positive mother can test positive for anti-HIV antibodies for up to 12-18months

Question 446

mx HIV

Answer 446

Prophylaxis against PCP, Avoidance of live oral polio vaccine and BCG, Antiretroviral therapy to suppress viral replication, Social, psychological, and family support

Question 447

chicken pox isolation

Answer 447

until all spots crusted

Question 448

isloation for impetigo

Answer 448

when crusted or 48h after starting abx

Question 449

isolation for measles

Answer 449

4d from start of rash

Question 450

islolation for mumps

Answer 450

5d from onset of swelling

Question 451

isolation for scabies

Answer 451

until after first tx

Question 452

isolation for scarlet fever

Answer 452

24h after starting abx

Question 453

isolation for whooping cough

Answer 453

48h after starting abx

Question 454

not requirijng isolation

Answer 454

conjunctivitis, glandular fever, hand foot and mouth, slapped cheek, head lice, threadowrms, tonsilits

Question 455

vaccines at birth

Answer 455

BCG if high risk

Question 456

vaccines at 8w

Answer 456

6 in 1 (diphtheria, whooping cough, polio, Hib, hep B, tetanus), oral rotavirus, Men B

Question 457

vaccines at 12w

Answer 457

6 in 1 (diphtheria, whooping cough, polio, Hib, hep B, tetanus), oral rotavirus, PCV

Question 458

vaccines at 16 w

Answer 458

6 in 1 (diphtheria, whooping cough, polio, Hib, hep B, tetanus), Men B

Question 459

vaccines at 12-13m

Answer 459

Hib/Men C, MMR, PCV, Men B

Question 460

vaccines 2-4y

Answer 460

4 in 1 (diphtheria, tetanus, whooping cough, polio), MMR

Question 461

vaccines 12-13y

Answer 461

HPV

Question 462

vaccines 13-18y

Answer 462

3 in 1 (tetanus, diphtheria, polio), Men ACWY

Question 463

presentation McCune ALbright syndrome

Answer 463

Characterised by the following triad of clinical features: Skin: hyperpigmented (café au lait) macules, Polyostic fibrous dysplasia, Autonomous endocrine gland hyperfunction (Ovary most commonly affected, Precocious puberty, Hyperthyroidism, Cushing’s)

Question 464

androgen insensitvity syndrome

Answer 464

spectrum of under-virilised phenotypes in the 46XY patient. (Patients who have a male karyotype but not clear male genitalia)
X-linked recessive condition due to end-organ resistance to testosterone causing genotypically male children (46XY) to have a female phenotype.

Question 465

features androgen insensitivity syndrome

Answer 465

‘primary amennorhoea’, undescended testes causing groin swellings, breast development may occur as a result of conversion of testosterone to oestradiol

Question 466

mx androgen insesnitivity syndrome

Answer 466

counselling - raise child as female, bilateral orchidectomy (increased risk of testicular cancer due to undescended testes), oestrogen therapy

Question 467

categories of undesccended testes

Answer 467

Undescended testes are subdivided into the following: Palpable undescended testes: usually at the external inguinal ring. These testes can be bought down into the scrotum with an orchidopexy performed through an inguinal incision. Impalpable testes: intra-abdominal, inside the inguinal canal, or absent. There is risk of malignant degeneration in an intraabdominal testis, need a Laparoscopy

Question 468

mx undescended testes

Answer 468

When to refer to surgeon: If still undescended at 3-6mths
Treatment: Orchidopexy at 6- 18 months of age.

Question 469

presentation testicualr torsion

Answer 469

Sudden onset severe scrotal pain, often associated with nausea and vomiting, Tender testis, Overlying scrotal skin may be reddened and oedematous.

Question 470

m testicular torsion

Answer 470

Immediate scrotal exploration is mandatory to salvage the testis, which should then be fixed to prevent recurrence.

Question 471

obesity

Answer 471

Obesity implies increased central (abdominal) fat mass, and can
be quantified using a number of clinical surrogate markers. BMI is the most convenient indicator of body fat mass.
Overweight: BMI >91st centile, wt <98th centile, Obese: BMI >98th centile

Question 472

RF for obesity

Answer 472

Parental/family history of obesity, Afro-Caribbean/Indian-Asian ethnic origins

Question 473

ause of obesity

Answer 473

Causes: ‘idiopathic’ obesity, Genetic predisposition (energy conservation), Increasingly sedentary lifestyle (energy expenditure), Increasing consumption and availability of high energy foods
Other underlying pathologies: Hypothyroidism, Cushing’s syndrome/disease, Growth hormone deficiency, Pseudohypoparathyroidism, Polycystic ovarian syndrome, Acquired hypothalamic injury e.g. CNS tumours, Prader-Willi syndrome, Bardet-Biedl syndrome, Myogenic causes: leptin deficiency (rare)

Question 474

complications of obesity

Answer 474

Psychological: low self-esteem, depression. ENT/ respiratory: obstructive sleep apnoea, obesity-hypoventilation syndrome, pulmonary hypertension. Orthopaedic: bowing of legs, slipped femoral epiphysis, osteoarthritis. Metabolic: impaired glucose tolerance/type 2 diabetes, hypertension, dyslipidaemia, PCOS. Hepatic: non-alcoholic steatohepatitis

Question 475

causes congenital hypothyeoidism

Answer 475

Thyroid dysgenesis: usually sporadic, resulting in thyroid aplasia/hypoplasia, ectopic thyroid (lingual/ sublingual). Thyroid hormone biosynthetic defect: hereditary e.g. Pendred’s syndrome. Iodine deficiency.. Congenital TSH deficiency

Question 476

features congenital hypothyroidism

Answer 476

Umbilical hernia, Prolonged jaundice, Constipation, Hypotonia, Hoarse cry, Poor feeding, Excessive sleepiness, Dry skin, Coarse faecies, Delayed neurodevelopment
Without early hormone replacement therapy, complications sequelae may occur: Neurodevelopmental delay and mental retardation, Poor motor coordination, Hypotonia, Ataxia, Poor growth and short stature

Question 477

mx congenital hypothyrodism

Answer 477

levothyrocine

Question 478

congenital adrenal hyperplasia

Answer 478

A rare family of disorders characterised by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens.
Deficiency of the 21alpha-hydroxylase enzyme is the most common form, accounting for over 90% of cases.

Question 479

presentation congenital adrenal hyperplasia

Answer 479

Classic CAH: includes a severe ‘salt wasting’ form that usually presents with acute adrenal crisis in early infancy (usually males at 7-10 days of life) and a ‘simple virilising’ form in which patients demonstrate masculinisation of the external genitalia (females at birth) or signs of virilisation in early life in males.
Non classic (late onset): Presents in females with signs and symptoms of mild androgen excess at or around the time of puberty.

Question 480

ix congenital adrenaal hyperplasia

Answer 480

Elevated plasma 17-hydroxyprogesterone levels, Elevated plasma 21-deoxycortisol levels, Increased urinary adrenocorticosteroid metabolites

Question 481

mx congenital adrenal hyperplasi

Answer 481

glucocorticoid replacement therapy – hydrocortisone. Salt-wasting form – mineralocorticoid therapy – fludrocortisone. Resistance to mineralocorticoid therapy – sodium chloride therapy. Urogenital surgery – performed in infancy in females with significant virilisation of the external genitalia.

Question 482

precocious ouverty classification

Answer 482

hypothalamic-gonadal axis, FSH and LH raised. Gonadotropin independent=due to excess sex hormones, FSH and LH low

Question 483

causes precocious puberty

Answer 483

Males: uncommon and usually organic cause. Bilateral testicular enlargement=gonadotropin release from intracranial lesion, unilateral testicular enlargement=testicular tumour, small testes=adrenal cause
Females: usually idiopathic or familial

Question 484

pathophysiology T1DM

Answer 484

chronic autoimmune condition. Immune tolerance is broken and antibodies against specific beta-cell autoantigens are generated (e.g. anti-islet cell, anti-insulin, anti-GluAD). T-cell activation leads to beta-cell inflammation (‘insulitis’) and to subsequent cell loss through apoptosis

Question 485

presnetation T1DM

Answer 485

: Weight loss, Polyuria, Polydipsia, Nocturia/nocturnal enuresis, Candida infection e.g. oral thrush

Question 486

diagnosis T1DM

Answer 486

Random >11.1mmol/L in a symptomatic child (x2 if asymptomatic)
Fasting >7.0mmol/L x2. Diabetes-related autoantibodies: islet cell antibody, anti-insulin antibody, anti-GluAD antibody, anti-IA-. OGTT peak >11.1 (2 hours after 75g oral glucose

Question 487

mx T1DM

Answer 487

: Education of child and family about diabetes, Insulin therapy, Screening for development of associated illness, Screening for diabetes-related microvascular complication
Blood glucose monitoring: A minimal testing frequency of 4 times per day should be encouraged

Question 488

presentation hypoglycaemia

Answer 488

Symptoms develop when blood glucose <3.5mmol/L. The frequency of hypoglycaemia is higher with more intensive insulin regimens and in young children.
ymptoms and signs include: Feeling of hunger, Sweatiness , Feeling faint/dizzy, ‘wobbly feeling’, Irritability/confusion/misbehaviour, Pallor

Question 489

mx hypoglycaemia

Answer 489

Acute episodes of mild to moderate symptomatic hypoglycaemia can be managed with oral glucose (glucose tablets or sugary drink). Oral glucose gels applied to the buccal mucosa can be used in the child who is unwilling or unable to cooperate to eat. Severe hypoglycaemia can be managed in the home with an IM injection of glucagon (1.0mg). This is available as a specific injection kit

Question 490

pathophysiology DKA

Answer 490

Caused by a decrease in effective circulating insulin associated with elevations in counter-regulatory hormones. This leads to increased glucose production by the liver and kidney and impaired peripheral glucose utilisation with resultant hyperglycaemia and hyperosmolality. Increased lipolysis, with ketone body production causes ketonemia and metabolic acidosis.

Question 491

presentation DKA

Answer 491

Hyperglycaemia and acidosis result in osmotic diuresis, dehydration, and obligate loss of electrolytes. Ketoacid accumulation also induces an ileus, resulting in nausea and vomiting and an exacerbation of the dehydration.

Question 492

mx DKA

Answer 492

Fluid, Insulin - one hour after fluid (reduce risk of cerebral oedema), Monitor glucose hourly, Monitor electrolytes, especially K+ ketones and ketones - 2 hourly, Hourly neuro observations
Watch out for: Cerebral oedema, Shock - extremely dehydration, Hypokalaemia, Aspiration - if DKA patient is vomiting make sure you put NG tube down and NBM, Thrombus

Question 493

Maturity onset diabetes of young (MODY

Answer 493

A clinical heterogenous group of disorders characterised by an autosomal dominant mode of inheritance, onset usually before the age of 25yrs, and non-ketotic diabetes at presentation.
The condition is due a primary defect in beta-cell function and insulin seceretion.

Question 494

guthrie screening test

Answer 494

9 things: CF, SC disease, congenital hypothyroidism, phenylketonuria (PKU), medium-chain acyl-CoA dehydrogenase deficiency (MCADD), maple syrup urine disease (MSUD), isovaleric acidaemia (IVA), glutaric aciduria type 1 (GA1), homocystinuria (pyridoxine unresponsive) (HCU)

Question 495

features suggesting dehydration

Answer 495

Appears unwell/deteriorating, Altered responsiveness (irritable, lethargic), Sunken eyes, Tachycardia, Tachypnoea, Reduced skin turgor, Dry mucous membranes (not reliable if the child is mouth breathing or just after a drink), Decreased urine output

Question 496

features suggesting shock

Answer 496

Decreased level of consciousness, Pale or mottled skin, Cold extremities, Pronounced tachycardia, Pronounced tachypnoea, Weak peripheral pulses, Prolonged capillary refill time, Hypotension

Question 497

maintenance fluids for child >28d

Answer 497

: isotonic crystalloids + 5% glucose (e.g. 0.9% sodium chloride + 5% glucose). 100 ml/kg/day for the first 10kg of weight, 50 ml/kg/day for the next 10kg of weight, 20 ml/kg/day for weight over 20kg

Question 498

maintenance fluids for neonate

Answer 498

The choice of fluid depends on the clinical situation: No critical illness=10% dextrose +/- additives, Critical illness(e.g. infantile respiratory distress syndrome, meconium aspiration)=seek expert advice (use fluids with no/minimal sodium initially). Birth to day 1: 50-60 ml/kg/day, Day 2: 70-80 mL/kg/day, Day 3: 80-100 mL/kg/day, Day 4: 100-120 mL/kg/day, Days 5-28: 120-150 mL/kg/day

Question 499

eplacement fluids

Answer 499

Use isotonic crystalloid that contains sodium with added glucose (e.g. 0.9% sodium chloride + 5% glucose). If there are ongoing losses (e.g. diarrhoea, vomiting) supplement with potassium (e.g. 10 mmol/L)
Calculate percentage dehydration: (well wt-current wt)/well wt x100
Fluid deficit (mL) = % dehydration x weight (kg) x 10
Total fluid requirement (mL) = maintenance fluids (mL) + fluid deficit (mL)

Question 500

resuscitation fluids

Answer 500

Glucose-free balanced crystalloids (e.g. Hartmann’s solution) are recommended as initial resuscitation fluids.
The standard fluid for resuscitation is 0.9% sodium chloride with no additives via intravenous (IV) or intraosseous (IO) access (if IV access is not possible) in a standard bolus of 10 mL/kg over <10 minutes.1
Exceptions to this rule in which smaller boluses may need to be used: Neonatal period (<28 days of age), Diabetic ketoacidosis, Septic shock, Trauma, Cardiac pathology (e.g. heart failure)
After the bolus has been administered, the volume status should be re-assessed (e.g. heart rate, respiratory rate, capillary refill time). If the patient is still shocked urgent senior advice should be sought.
Maintenance fluids after resuscitation: There is no need to subtract the resuscitation boluses from the total 24-hour fluid requirements. For a shocked child, we assume 10% dehydration based on body weight.

Question 501

anaemia

Answer 501

Hb level below the normal range. The normal range varies with age, so anaemia can be defined as: Neonate=Hb less than 140g/L, 1 -12 months=Hb less than 100g/L, 1-12 years=Hb less than 110g/L

Question 502

auses microcytic anaemia

Answer 502

low MCV): Iron deficiency anaemia, B-thalassaemia trait (usually children of Asian/ Arabic / Med origin), Anaemia of chronic disease e.g. CKD

Question 503

RF iron deficiency anaemia

Answer 503

Preterm, LBW infants, multiple births, Adolescent females (growth spurt and menstruation), Low iron-containing diet due to poverty, fad diets, or strict vegans

Question 504

presentation iron deficiency anaemia

Answer 504

Most children asymptomatic until the Hb drops below 60g/L -70g/L., As anaemia worsens – children tire easily, young infants feed slower, Ask about blood loss, Ask about signs/symptoms relating to malabsorption, May appear pale – pallor unreliable sign unless pallor of the conjunctivae, tongue or palmar creases, ‘pica’ – inappropriate eating of non-food materials e.g. soil: evidence ID anaemia may be detrimental to behaviour and intellectual function.

Question 505

mx iron deficiency anaemia

Answer 505

Dietary advice and supplementation with oral iron, Iron supplements should be continued until Hb is normal + min. 3 months to replenish iron stores.

Question 506

causes vit B12 deficeincy

Answer 506

Vegans/ vegetarians are at risk. Alternatively, can have defective absorption due to intrinsic factor deficiency, defective B12 transport, intestinal disease causing malabsorption (ileal resection, IBD, coeliac disease), or bacterial over-growth in small bowel.

Question 507

causes folate deficiency

Answer 507

Malnutrition (marasmus, kwashiorkor), goat’s milk feeding. Malabsorption, e.g. coeliac disease, IBD. Increased requirements e.g. rapid growth, chronic haemolytic anaemias. Drugs e.g. phenytoin, valproate, trimethoprim, nitrofurantoin. Disorders of folate metabolism

Question 508

presentation anaemia due to B12/folate defieincy

Answer 508

Insidious onset of pallor, fatigue, anorexia, glossitis, developmental delay and hypotonia, In severe cases, subacute combined degeneration of cord (rare in children): paraesthesia of hands/feet, ataxic gait, loss of vibration sense

Question 509

mx B12/folate defieicny

Answer 509

Improve diet, B12 deficiency: IM hydroxocobalamin, Folate deficiency: daily oral folic acid

Question 510

membrnaopathies

Answer 510

Autosomal dominant conditions which result in an abnormally shaped red cell. Deficiency of red cell membrane proteins caused by a variety of genetic lesions

Question 511

Hereditary spherocytosis

Answer 511

Autosomal dominant , Shortage of red blood cells , Less deformable spherical RBCs, so trapped in spleen -> extravascular haemolysis. Signs: Splenomegaly, Jaundice

Question 512

Hereditary elliptocytosis

Answer 512

Autosomal dominant, Abnormally large number of the erythrocytes are elliptical. Mostly asymptomatic

Question 513

Glucose-6-phosphate dehydrogenase (G6PD) deficiency:

Answer 513

X-linked recessive, Most are asymptomatic, Lack of enzyme that maintains protective protein against oxidant injury. In attacks – rapid anaemia and jaundice. Precipitants – drugs, exposure to broad beans, illness, henna, Drugs = primaquine, sulphonamides, quinolones, dapsone, nitrofurantoin. Tests=Enzyme assay, Film

Question 514

Pyruvate kinase deficienc

Answer 514

Autosomal recessive, Decreased ATP production causes decreased RBC survival. Clinical features=Variable chronic haemolysis, Prone to aplastic crisis in Parvovirus B19 infection.

Question 515

B thalassaemia types

Answer 515

Characterised by a severe reduction in the production of B-globin. Disease severity depends on the amount of residual HbA and HbF production. Beta-Thalassaemia major – most severe form. HbA cannot be produced. Beta-Thalassaemia minor - A carrier state that is usually asymptomatic. Beta-Thalassaemia intermedia – milder and variable severity

Question 516

presentation B thalassaemia

Answer 516

Severe anaemia which is transfusion dependent 3-6months, Jaundice , Faltering growth/ growth failure, Failure to feed, Extramedullary haemopoiesis – develop hepatosplenomegaly and bone marrow expansion (leads to maxillary overgrowth and skull bossing).

Question 517

mx B thalassaemia

Answer 517

regular blood transfusions, Iron chelation – SC desferrioxamine or an iron chelator drug e.g. deferasirox, Bone marrow transplant – only cure.

Question 518

alpha thalassaemia types

Answer 518

If all 4 genes are deleted, death is in utero – Bart’s hydrops. If 3 genes are deleted (–/-), HbH disease occurs – moderate anaemia and features of haemolysis – hepatosplenomegaly, leg ulcers, jaundice. If 2 genes are deleted (–/) – asymptomatic carrier state – decreased MCV. If one gene is deleted – normal clinical state.

Question 519

sickle cell anaemia forms

Answer 519

Sickle cell anaemia (HbSS) – homozygous HbS = mutation in both beta-globin genes, HbSC disease (HbSC) – HbS and HbC = no normal (HbA) beta-globin genes, Sickle beta-thalassaemia – HbS and beta-thalassaemia , Carriers (sickle trait) – HbS and one normal beta-globin gene.

Question 520

pathogenessi SC anaemia

Answer 520

deform the red cells into a sickle shape. These have a reduced lifespan and may be trapped in microcirculation resulting in vaso-occlusion = ischaemia in an organ or bone.

Question 521

features SC anaemia

Answer 521

Asymptomatic, Anaemia + clinically detectable jaundice, Increased susceptibility to infection, Painful (vaso-occlusive) crises – occlusions causing pain with varying frequency and severity, Priapism (persistent and painful erection of the penis), Splenomegaly

Question 522

long term problems in SC anaemia

Answer 522

Short stature and delayed puberty, Stroke and cognitive problems, Adenotonsillar hypertrophy, Cardiac enlargement, Heart failure, Renal dysfunction, Pigment gallstones, Leg ulcers, Psychosocial problems – education and behaviour

Question 523

mx acute SC crises

Answer 523

Analgesia, Antibiotics: broad spectrum cephalosporin, after blood culture if fever >38°C, Oxygen, Blood: transfusion for aplastic crisis, sequestration or anaemia.

Question 524

mx SC

Answer 524

vaccinations and oral penicillin should be given daily throughout childhood. OD oral folic acid. Avoid exposure to cold, dehydration, excessive exercise, undue stress, hypoxia (precipitating factors). Bone marrow transplant is the only cure.Treatment of chronic problems – hydroxycarbamide, which increases HbF production and helps protect against further crise

Question 525

Rh disease

Answer 525

occurs when a maternal antibody response is mounted against foetal red cells. These immunoglobulin (IgG) antibodies cross the placenta and cause foetal red blood cell destruction. The ensuing anaemia, if severe, precipitates foetal hydrops, which is often referred to as immune hydrops.

Question 526

mx Rh diseease

Answer 526

Close antenatal supervision +/- intrauterine blood transfusion. If treatment required, oral folic acid 250mcg/kg/day for 6 months

Question 527

causes aplastic anaemia

Answer 527

Autoimmune, Drugs, Viruses – parvovirus, hepatitis, Irradiation, Inherited – Fanconi anaemia

Question 528

fanconi anaemia

Answer 528

Autosomal recessive in FANC genes.
This rare condition leads to progressive bone marrow failure affecting all 3 haemopoietic cell precursors

Question 529

presentation fanconi anaemia

Answer 529

4-10yrs, bruising and purpura or insidious onset anaemia. Congenital abnormalities – short stature, abnormal radii and thumbs, renal malformations, microphthalmia, pigmented skin lesions

Question 530

mx fanconi anaaemia

Answer 530

Supportive e.g. RBC transfusion, hearing aids, orthopaedic. Immunosuppression with corticosteroids and androgens (oxymetholone). Bone marrow transplantation

Question 531

haemophilia

Answer 531

Haemophilia A = FVIII deficiency. Haemophilia B = FIX deficiency.

Question 532

presentation haemophilia

Answer 532

Recurrent spontaneous bleeding into joints and muscles – can lead to crippling arthritis. Intracranial bleeds – usually follows minor head trauma.

Question 533

mx haemophilia

Answer 533

Haemophilia A Management: Avoid NSAIDs and IM injections (including vitamin K at birth). Prophylaxis: alternate day IV FVIII concentrates to prevent spontaneous bleeds. Desmopressin raises factor VIII levels
Haemophilia B: Treat with recombinant factor IX.

Question 534

von willebrnad disease

Answer 534

An inherited bleeding tendency caused by a quantitative or qualitative deficiency of vWF.
Inheritance is usually autosomal dominant. Type 1 is most common and usually mild.

Question 535

presentation von willebrand disease

Answer 535

Bruising, Excessive, prolonged bleeding after surgery, Mucosal bleeding such as epistaxis and menorrhagia.

Question 536

diagnosis von willebrand disease

Answer 536

: APTT is usually increased, vWF antigen levels reduced and function decreased

Question 537

mx von willebrna ddisease

Answer 537

Type 1 – usually treated with DDAVP, which causes secretion of FVIII and vWF into plasma. More severe types – plasma-derived FVIII concentrate.

Question 538

immune thrombocytopeniea

Answer 538

caused by IgG autoimmune antibody to platelet cell membrane antigens leading to platelet destruction in the spleen and liver.

Question 539

ntation ITP

Answer 539

Most present between 2-5yrs, but can occur at any age, Many have preceding viral infection e.g. URTI, Bruising, petechiae, purpura. Mucosal bleeding e.g. bleeding from gums, Major haemorrhage uncommon, Physical examination otherwise usually normal – no splenomegaly

Question 540

mx ITP

Answer 540

only if symptoms are severe. oral prednisolone, IV anti-D or IV immunoglobulin. These all have significant side effects

Question 541

chronic ITP

Answer 541

platelet count remains low after 6mths = chronic. Treatment: mainly supportive. Children with chronic persistent bleeding are rare and require specialist car

Question 542

classification of CNS tumours

Answer 542

nfratentorial tumours (>50%): present with raised ICP, headaches and vomiting, and cerebellar ataxia. Supratentorial tumours: present with raised ICP, focal neurology, hypothalamic/pituitary dysfunction, and visual impairment. Primary spinal tumours (rare): differential diagnosis includes astrocytomas and ependymomas.

Question 543

sentation brain tumour

Answer 543

cord compression. CNS metastases: of extracranial tumours (Rare)

Question 544

mx brain tumur

Answer 544

Initial management: Diagnostic imaging = CT and MRI
Raised intracranial pressure: high dose steroids (usually dexamethasone), CSF drainage: initial surgery may involve CSF diversion only, biopsy, or complete resection, depending on location and likely diagnosis.
Treatment: excision and craniospinal radiotherapy. Additional chemotherapy

Question 545

craniopharyngioma

Answer 545

Slow-growing midline epithelial tumours in the suprasellar area from ‘Rathke’s pouch’

Question 546

presentation retinoblastoma

Answer 546

absent or abnormal light reflex (leukocoria), squint, or visual deterioration
Sporadic or familial (40%) forms that are unilateral or bilateral (30%) on presentation
Peak incidence: unilateral disease, 2-3yrs; bilateral disease, 0-12 months

Question 547

mx retinoblastoma

Answer 547

surgery, chemotherapy, and focal therapy

Question 548

wilms tumour

Answer 548

embryonal tumour of the kidney

Question 549

presentation wilms tumour

Answer 549

Mostly as a visible or palpable abdominal mass. Usually painless. Haematuria and hypertension may also be seen

Question 550

mx wilms tumour

Answer 550

Surgical excision required, Chemotherapy is used for all tumours.

Question 551

hepatoblastoma mx

Answer 551

Chemotherapy including platinum drugs and anthracyclines for HBL. Good surgical result is critical for long-term survival.

Question 552

neuroblastoma

Answer 552

A malignant embryonal tumour derived from neural crest tissue with a wide spectrum of behaviour

Question 553

presetnation neuroblastoma

Answer 553

Non-specific and variable. Palpable mass (may be painless), Compression of nerves (e.g. Horner’s, spinal cord), airway, veins, bowel, Bone: pain and/or limp, Lymphadenopathy and signs of pancytopenia , Sweating, pallor, water diarrhoea, and hypertension

Question 554

ix neuroblastoma

Answer 554

: Urine catecholamine (VMA or homovanillic acid (HVA)) to creatinine ratio,131I-MIBG uptake scan: usually +ve

Question 555

mx neuroblastoma

Answer 555

Biological factors, such as MYCN amplification and 17q gain. Completely resected localised neuroblastoma may need no further treatment

Question 556

osteosarcoma presetnation

Answer 556

Localised pain and swelling, Pathological fracture, Rarely – erythema. Most affect the long bones around the knee and humorous. The metaphysis is a more common site than mid-shift..

Question 557

mx osteosarcoma

Answer 557

Chemotherapy -> surgery -> chemotherapy.

Question 558

presentation ewings sarcoma

Answer 558

Localised pain and swelling, Sometimes pathological fracture, Diaphysis of long bones is more commonly affected than metaphysis. The axial skeleton is involved more often than in OS with the pelvis the most common site

Question 559

mx ewingd ssarcoma

Answer 559

Chemotherapy -> surgery -> chemotherapy.

Question 560

presentation rhabdomyosarcoma

Answer 560

Mass, pain and obstruction of: Bladder, Pelvis, Nasopharynx, Parameningeal, Paratestis, Extremity, Orbit, Intrathoracic. Lymph node involvement is common

Question 561

mx rhabdomyosarcoma

Answer 561

Chemotherapy (6-9 courses) with ifosfamide or cyclophosphamide, actinomycin, vincristine, anthracyclines. Surgery is reserved for accessible sites (paratesticular, peripheral) after 3-6 courses of chemotherapy). Radiotherapy after surgery for residual tumour and alveolar histology.

Question 562

ALL

Answer 562

This is the most common malignancy in childhood. It arises from malignant proliferation of ‘pre-B’ (common ALL) or T-cell lymphoid precursors

Question 563

presentation ALL

Answer 563

Typically with a short history (days or weeks), and with symptoms and signs reflecting pancytopenia, bone marrow expansion, and lymphadenopathy. Petechiae, Bruising, Pallor, Tiredness, Bone/joint pain/swelling , Limp, Lymphadenopathy, Airway obstruction, Pleural effusion

Question 564

ix ALL

Answer 564

Bone marrow, CSF for cytospin, Characteristic blast cells on blood film and bone marrow. WCC usually high, CXR and CT scan to look for mediastinal and abdominal lymphadenopathy, Lumbar puncture should be performed to look for CNS involvement

Question 565

ALL

Answer 565

Induction (4wks), Consolidation CNS-directed therapy, Maintenance (2y for F, 3 M). Involves chemo and steroids

Question 566

poor prognostic factors ALL

Answer 566

Male gender, Age <2yrs or >10yrs, High WCC at diagnosis, Philadelphia chromosome -t(9;22), MLL gene rearrangements (e.g. t(4;11) etc. poor response to induction and failure to remit by day 28.

Question 567

AML

Answer 567

from malignant proliferation of myeloid cell precursors

Question 568

presentation AML

Answer 568

Symptoms and signs of bone marrow replacement (Pallor, Tiredness, Breathlessness, Frequent infections, Having a high temp, sweating a lot) Gum hypertrophy, Splenomegaly, Lymphadenopathy less prominent than ALL, Intrathoracic extramedullary disease less common than ALL, Solid deposits (chloroma) occasionally seen.

Question 569

ix AML

Answer 569

WCC is often increased, Bone marrow biopsy – AML differentiated from ALL by Auer rods.Immunophenotyping

Question 570

genetic conditions predisposing to AML

Answer 570

Fanconi syndrome, Blood syndrome, Ataxia telangiectasia, Kostmann’s syndrome, Diamond-Blackfan syndrome, Klinefelter’s, Turner’s syndrome, Neurofibromatosis, Incontinentia pigmenti

Question 571

mx AML

Answer 571

4 courses intensive myeloablative chemotherapy. Supportive measures: Blood/platelet transfusion, IV fluids, Hickman line for IV access. Bone marrow transplant

Question 572

CML

Answer 572

Classically associated with Philadelphia chromosome +ve disease t(9;22). Rare

Question 573

lymphoma

Answer 573

A malignant growth of white blood cells – predominantly the lymph nodes but also found in the blood, bone marrow, liver and spleen.
M > F

Question 574

ix hodgkins lumphoma

Answer 574

Histology shows characteristic cells with mirror-image nuclei are found, called Reed-Sternberg.
Lymph node excision biopsy if possible. CXR, CT/PET of neck, chest, abdo and pelvis

Question 575

presentation hodgkins lymohoma

Answer 575

Enlarged, non-tender, rubbery superficial lymph nodes. Constitutional upset – fever, weight loss, night sweats, pruritus, lethargy. Alcohol-induced lymph pain, Lymphadenopathy (abnormal lymph nodes, Cachexia (weakness and wasting of the body due to severe chronic illness, Anaemia, Spleno/hepatomegaly

Question 576

staging hodgkins lymphoma

Answer 576

Staging – Ann-Arbor
I. Confined to single lymph node region
II. Involvement of two or more nodal areas on the same side of the diaphragm
III. Involvement of nodes on both sides of the diaphragm
IVSpread beyond the lymph nodes on both sides of the diaphragm
Each stage is either ‘A’ (no systemic symptoms) or ‘B’ (presence of B symptoms) – weight loss, unexplained fever, night sweats. B indicates worse disease.

Question 577

mx hodgkins lymphoma

Answer 577

chemotherapy. Stages IA-IIA: radiotherapy + short courses of chemotherapy. Stages IIA-IVB (with >3 areas involved): longer courses of chemotherapy

Question 578

causes NHL

Answer 578

: Immunodeficiency-Drugs, HIV . Infection -Infection from EBV transform cells, Helicobacter pylori

Question 579

classifciation NHL

Answer 579

: Lymphoblastic , Mature B cell (Burkitt or Burkitt-like), Large cell lymphoma
NHL staging – St Jude system

Question 580

staging NHL

Answer 580

NHL staging – St Jude system
Stage I: single site or nodal area (not abdomen or mediastinum)
Stage II: regional nodes, abdominal disease
Stage III: disease on both sides of the diaphragm
Stage IV: bone marrow or CNS disease

Question 581

presentation NHL

Answer 581

Superficial lymphadenopathy , Extra-nodal disease, Gut - Gastric MALT (caused by H.pylori)=Dyspepsia , Weight loss, Dysphagia . Small bowel lymphomas=Diarrhoea, Vomiting , Abdominal pain , Oropharynx = Waldeyer’s ring lymphoma causes obstructed breathing. Systemic features=Fever, Night sweats , Weight loss, Pancytopenia from marrow involvement (anaemia, bleeding)

Question 582

mx NHL

Answer 582

Low-grade lymphomas (indolent)=often incurable, radio, chemo, rituximab. High-grade lymphomas (aggressive)= ‘R-CHOP’ chemotherapy regimen: rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, prednisolone.

Question 583

karyotype klinefelters

Answer 583

47 XXY

Question 584

presentation klinefelters

Answer 584

hypergonadotrophic hypogonadism with decreased testosterone production. Tall and may develop feminine body build. Testes are small and men are generally infertile (azoospermia). Gynaecomastia develops at puberty, Reduced secondary sexual hair

Question 585

problems associated with klinefelters

Answer 585

Type 2 diabetes, osteoporosis, Cardiovascular diseaseandblood clots, AI disorders such aslupus, hypothyroidism, Anxiety, learning difficultiesanddepression, Male breast cancer

Question 586

mx klinefelters

Answer 586

Testosterone replacement therapy once puberty starts

Question 587

karyotype for turners syndrome

Answer 587

45 XO

Question 588

presentation turners syndrome

Answer 588

At birth oedema of dorsa of hands, feet and loose skinfolds at the nape of the neck, Webbing of neck, low posterior hairline, small mandible, prominent ears, epicanthal folds, high ached palate, broad chest, cubitus valgus, hyperconvex fingernails, Hypergonadotrophic hypogonadism, streak gonads, Recurrent otitis media, Ptosis, Low set ears, Wide carrying angle at elbows, Widely spaced hypoplastic nipples, Low posterior hairline, Excessive pigmented naevi, Hypothyroidism, Puffiness of the hands and feet is a common neonatal finding

Question 589

provlems asssociated with turners

Answer 589

CHD (esp. CoA and VSD)
Bicuspid aortic valve, coarctation of the aorta, Structural renal abnormalities e.g. horseshoe kidney, Hypoplastic ovaries (1° amenorrhoea and infertility)

Question 590

mx turners syndrome

Answer 590

Daily SC injections of high dose recombinant human growth hormone, oestrogen

Question 591

karyotype down syndrome

Answer 591

Trisomy 21 = due to non-disjunction during maternal oogenesis, Robertsonian translocation, mosaicism

Question 592

presentation down syndrome

Answer 592

Generalised hypotonia and marked head lag – FLOPPY BABY, small low-set ears, up-slanting eyes, prominent epicanthic folds, a flat facial profile , protruding tongue, Brushfield’s spots apparent in the iris (whitish spots), Flat occiput (brachycephaly) and short neck, Short broad hands (brachydactyly), short incurved little fingers (clinodactyly), single transverse palmar crease, and a wide ‘sandal’ gap between the first and second toes, Mildly short stature, Intellectual impairment

Question 593

problems associated with down syndrome

Answer 593

Congenital heart disease – ASD, AVSD, VSD, ToF, GI problems – duodenal atresia, anal atresia, HSD, Increased risk of infection, Developmental hip dysplasia, Eczema, Deafness: both sensorineural and conductive, Cataracts, Leukaemia, Acquired hypothyroidism

Question 594

mx down syndrome

Answer 594

Routinely test TFT annually, Refer for audiology and ophthalmic assessment 1-2 yearly, monitor for alzhemiers

Question 595

karyotype edwards syndrome

Answer 595

trisomy 18

Question 596

presentation edwards syndrome

Answer 596

Babies are usually SGA,
Congenital heart disease – VSD +/- valve dysplasia, Short sternum, Overriding fingers, ‘rocker-bottom’ feet
Median life expectancy is 4 days

Question 597

karyotype pataues syndrome

Answer 597

trisomy 13

Question 598

presentation pataus syndrome

Answer 598

Holoprosencephaly,
SGA, Microcephalic, Microphthalmia,
Cleft lip/palate, Congenital heart disease e.g. ASD or VSD, Renal anomalies e.g. fused kidneys, Postaxial polydactyly
Majority die within first yr

Question 599

heritance kallmans

Answer 599

X-linked recessive

Question 600

presentation kallmans

Answer 600

hypogonadotropic hypogonadism: ‘Delayed puberty’, cryptorchidism, Anosmia, Sex hormone levels are low, LH, FSH levels are inappropriately low/normal, normal or above average height

Question 601

heritance fragil X syndrome

Answer 601

Full expansion CGG repeats, more M

Question 602

eatures fragil X

Answer 602

A large forehead or ears, with a prominent jaw, An elongated face, Protruding ears, forehead, and chin, Loose or flexible joints, Flat feet, Developmental delays, stutter
intellectual and learning disabilities,

Question 603

oblems associated with fragile X

Answer 603

ASD, Impulsiveness, Attention difficulties, hyperactivity, Mitral valve prolapse

Question 604

inheritance angelmans syndrome

Answer 604

impaired or absent function of the maternally imprinted UBE3A gene on chromosome 15q11.13

Question 605

atures angelmans

Answer 605

Unprovoked laughing/clapping, microcephaly, seizures, ataxia, mental retardation, fascination with water

Question 606

heritance praderwilli

Answer 606

disruption to the paternally derived imprinted domain on 15q11-13

Question 607

presentation praderwilli

Answer 607

Floppy baby, rapid wt gain 1-6y, An excessive appetite and overeating, Restricted growth(children are much shorter than average), hypotonia, Learning difficulties, Lack of sexual development, Behavioural problems,

Question 608

problems assocaited with prader willi

Answer 608

Type 2 diabetes, Heart failure, Respiratory difficulties

Question 609

inheritance noonans

Answer 609

autosomal dominant

Question 610

presentation noonans

Answer 610

Short stature, hypertelorism, ptosis, ear abnormalities, broad neck, Congenital heart disease (esp. pulmonary stenosis), Cardiomyopathy , pectus carinatum superiorly and pectus excavatum inferiorly, Mild developmental delay, Undescended testes

Question 611

nheritance williams sndrome

Answer 611

microdeletion on chromosome 7q11 – elastin gene

Question 612

preesntation wiiliams syndrome

Answer 612

cardiac defect – supravalvular aortic stenosis, Peri-orbital features, Full cheeks, Anteverted nares, Wide mouth with full lips, Small widely spaced teeth, mild mental retardation, over-friendliness, short attention span, anxiety
Some infants may have hypercalcaemia

Question 613

oblems associated with williams syndrome

Answer 613

Avoid taking extra calcium and vitamin D - Calcium deposits can cause kidney problems and narrowed blood vessels can cause heart failure.

Question 614

nheritance marfan syndrome

Answer 614

variable austosomal dominant

Question 615

presentation marfans syndrome

Answer 615

Ghent criteria (no family history) = 2 major and 1 minor: Major criteria can include: An enlarged aorta, A tear in the aorta, Dislocation of the lens of the eye, A family history, At least 4 skeletal problems, such asflat feetor acurved spine (scoliosis), Enlargement of the lining that surrounds part of the spinal cord (dural ectasia)
Minor criteria can include: Short-sightedness (myopia), Unexplained stretch marks, Loose joints, A long, thin face, A high, arched palate

Question 616

causes of limp 0-5y

Answer 616

discitis, DDH, Transient synovitis, Septic arthritis, osteomyelitis, septic arthritis

Question 617

causes oflimp 5-10y

Answer 617

discitis, Transient synovitis, Septic arthritis, Perthes’ disease, osteomyelitis, Discoid meniscus, septic arthritis, Kohler’s disease, Freiberg’s disease, Tarsal coalition, Verruca

Question 618

causes of limp 10-15y

Answer 618

discitis, Slipped upper femoral epiphysis, Septic arthritis, osteomyelitis, Osgood-Schlatter’s disease, Osteochondritis dessicans, Patellofemoral pain syndrome, Chondromalacia patella, Sever’s disease, Tarsal coalition, Verruca, Ingrowing toenail

Question 619

causes septic arthritis

Answer 619

75% are in the lower limb. Knee > hip > ankle. 25% upper limb.. <12mths: Staph. Aureus, group B streptococcus, gram -ve bacilli, Candida albicans. 1-5yrs: Staph aureus, haemophilus influenza, Group A strep, Strep. Pneumoniae, Neisseria gonorrhoeae (child abuse). 5-12yrs: Staph aureus, Group A strep. 12-18yrs: Staph aureus, Neisseria gonorrhoeae (sexually active)

Question 620

preseentation septic arthritis

Answer 620

Decreased range of movements or pseudo paralysis, Pain on passive motion, Hot, warm, swollen joints, Inability to weight bear, Systemic symptoms of infection

Question 621

ix septic arthritis

Answer 621

blood cultures, X-ray of joint= joint space narrowing and erosive changes = later signs, Joint aspiration

Question 622

mx septic arthritis

Answer 622

IV abx, after aspirate taken, for up to 3wks, followed by oral abx for 4-6wks. irrigation and debridement of affected joint, splint in position of function

Question 623

cause osteomyelitis

Answer 623

Staphylococcus aureus is most common in children in all age groups. Neonates: group B streptococcus and Gram -ve enteric bacilli, <2yrs: Haemophilus influenzae (rare), >2yrs: Gram +ve cocci, Pseudomonas aeruginosia. Adolescents: Neisseria gonorrhoea

Question 624

presentation osteomyelitis

Answer 624

Pain, Limping , Refusal to walk/weight bear, Fever, Malaise , Flu-like symptoms, Overlying bone may be tender, with/without swelling. Long bones principally affected: Tibia > femur > humerus

Question 625

ix osteomyeliti

Answer 625

blood cultures, XR, MRI, aspiration

Question 626

mx osteomyelitis

Answer 626

IV abx for a min. of 2wks, followed by oral abx for 4wks. Usually flucloxacillin. Drainage and debridement may be needed

Question 627

RF DDH

Answer 627

: Family history, Female, Racial predilection, Breech presentation

Question 628

screening for DDH

Answer 628

Ortolani’s test=If hip is dislocation, gently elevate and abduct the dislocated hip to reduce it (clunk of reduction), Barlow’s test= If hip isn’t dislocated, can it be – gently adduct and depress femur – vulnerable hip dislocates.

Question 629

mx DDH

Answer 629

: Age <6 months: Pavlik harness (maintain hip in flexed position with some hip abduction), Age 6-18 months: manipulation and closed reduction + hip spica plaster cast

Question 630

complications DDH

Answer 630

redislocation, residual acetabular dysplasia; avascular necrosis, early osteoarthritis changes

Question 631

perthes disease

Answer 631

avascular necrosis occurs.

Question 632

RF perthes disease

Answer 632

Boys, 4-10 years, Family history, Low birth weight, Delayed skeletal maturity

Question 633

sx perthes disease

Answer 633

Mild/intermittent anterior thigh/groin/referred knee pain with limp, Classical ‘painless limp’, decreased hip ROM

Question 634

ix perthes disease

Answer 634

XR, Technetium 99 bone scan

Question 635

mx perthes disease

Answer 635

T<6 years: Supportive (NSAIDs, physiotherapy) surgical >6y

Question 636

disease associated with slipped upper femoral epiphysis (slipped capital femoral epiphysis)

Answer 636

Associated with: hypothyroidism, hypogonadism, renal osteodystrophy, retroversion of femoral neck or vertical growth plate, Down syndrome, radiotherapy/ chemotherapy

Question 637

sx slipped upper femoral epiphysis

Answer 637

Groin, thigh, or knee pain, Antalgic gait, Limited hip flexion and abduction, flexion into external rotation and thigh atrophy, obese, 10-15y

Question 638

ix slipped upper femoral epiphysis

Answer 638

XR AP and lateral (typically frog-leg)views are diagnostic

Question 639

mx slipped upper frmoral epiphysis

Answer 639

pin, internal fixation

Question 640

genu valgum

Answer 640

knock knees

Question 641

causes genu valgum

Answer 641

Causes: physiological, rickets, congenital dislocation of patella, myelodysplasia

Question 642

ix genu valgum

Answer 642

AP and lateral full-length X-rays

Question 643

mx genu valgum

Answer 643

none, surgery if severe. Usually resolves

Question 644

genu varum

Answer 644

bowed knees

Question 645

causes genu varum

Answer 645

physiological (curled up in utero), osteogenesis imperfecta, vitamin D/calcium deficiency, metaphyseal dysplasia, achondroplasia, physeal injury, osteochondromas

Question 646

ix genu varum

Answer 646

weight bearing X-ray AP and lateral lower leg views

Question 647

mx genu varum

Answer 647

: if severe – treat by guided growth using staples or eight plates, usually resolves

Question 648

osteochondroses

Answer 648

a spectrum of conditions primarily affecting the epiphyses, but may also involve cartilage and bone. Not always due to inflammation, and may be due to trauma or over-usage, vascular irregularities or may be a normal variation.

Question 649

osgood slatters disease

Answer 649

Tibial tubercle traction apophysitis

Question 650

sx osgood slatters

Answer 650

Boys (age 12-14yrs) > girls (aged 10-12yrs), Presents with painful swelling over a prominent tibial tubercle (usually unilateral), associated with running/jumping. Often presents in sporty teenagers.

Question 651

ix osgood slatters

Answer 651

An irregular fragmented tibial tubercle may be seen on XR

Question 652

mx osgood slatters

Answer 652

usually self limiting. activity modification, rest, ice, knee brace, NSAIDs, surgery

Question 653

sx discoid meniscus

Answer 653

asymptomatic, instability, clicking, pain

Question 654

iagnosis discoid meniscus

Answer 654

MRI, arthroscopy

Question 655

mxdiscoid meniscus

Answer 655

none if asx, meniscectomy

Question 656

transient synovitis

Answer 656

inflammation in the hip. It occurs when a viral infection, such as an upper respiratory infection, moves to and settles in the hip joint

Question 657

sx transietn synovitis

Answer 657

Unilateral hip or groin pain is the most common symptom reported, Recent history of an upper respiratory tract infection,pharyngitis,bronchitis, orotitis media, pain on movement, reduced abduction and internal rotation

Question 658

mx transietn synovitisq

Answer 658

Bedrest for 7-10 days, no weight bearing on the affected limb. Apply heat and massage.NSAIDs – ibuprofen/ naproxen, for pain relief

Question 659

classification JIA

Answer 659

Systemic arthritis, Oligoarthritis (most common), Polyarthritis (RF +ve), Polyarthritis (RF -ve), Psoriatic arthritis , Enthesitis-related arthritis , Undifferentiated arthritis

Question 660

sx JIA

Answer 660

Limp, stiffness and loss of function, pain or malaise, Gradual onset, Inflammatory symptoms worse after rest or inactivity, Associated rash, fever, weight loss, Sore throat, URTI, antecedent infections, and travel, FHx of arthritis, psoriasis, colitis, rheumatic fever or acute iritis

Question 661

oligoarticular JIA

Answer 661

<4 joints

Question 662

psoriatic JIA

Answer 662

psoraissia nd arhtritisq

Question 663

Enthesitis related arthritis:

Answer 663

Typically presents as plantar fasciitis

Question 664

systemic JIA

Answer 664

Present very unwell, Rashes (worse during fever), Wide spread lymphadenopathy, Hepatosplenomegaly, Build up fluids - pleural effusions, ascites

Question 665

ix JIA

Answer 665

FBC may show mild anaemia and thrombocytosis, Neutrophilia suggests sepsis or systemic JIA, ESR/CRP usually normal or mildly elevated. Rheumatoid factor: non-specific test, but significant in polyarthritis, ANA: non-specific. In oligoarticular JIA limited prognostic determinant for iritis.

Question 666

mx JIA

Answer 666

physio, NSAIDS, steroids, immunotherapy (methotrexate, TNF-a inhibitors)

Question 667

complications JIA

Answer 667

flexion contractures (requiring physio and splints), joint destruction (requiring prostheses at young ages), growth failure (from steroids and chronic disease) and chronic anterior uveitis (causing visual impairment).

Question 668

back pain reed flags

Answer 668

Several weeks of symptoms, Night pain, Increasing symptoms, Abnormal neurology, Recurrent onset of scoliosis, Night sweats

Question 669

discitis

Answer 669

Inflammation of the disc space:

Question 670

ntation discitis

Answer 670

: fever, irritability, unwilling to walk, back pain, abdo pain, mainly infants and children

Question 671

ix discitisi

Answer 671

bloods – raised ESR/CRP, MRI, bone scan

Question 672

mx discitis

Answer 672

abx

Question 673

kyphosis

Answer 673

increased curvature of the spine in the sagittal plane, visible from the side

Question 674

presentation kyphosis

Answer 674

hyperextension – stand, bend forwards, bend backwards, associated lumbar lordosis, limited straight leg raising
Postural kyphosis: Flexible, usually painless, onset <10 years. Usually tall, flat footed.
Congenital kyphosis: Rigid, occasionally painful, onset <10 years.

Question 675

ix kyphosis

Answer 675

PA and lateral standing X-rays of entire spine

Question 676

mx kyphosis

Answer 676

Postural kyphosis: physiotherapy
Congenital kyphosis: brace, if progression fusion to prevent paraplegia

Question 677

RF scoliosis

Answer 677

FHx – daughters of affected mothers are more likely to be affected, Marfans, Neurofibromatosis

Question 678

classification scoliossi

Answer 678

True idiopathic scoliosis: Painless, convex to the right in the thoracic spine, not associated with any neurological changes. Treatment – observation -> manipulation + casting -> surgery
Congenital scoliosis: Abnormal vertebral development in the first trimester, Often needs surgery
Scoliosis secondary to neuromuscular disorders: Progresses more rapidly and may continue after maturity

Question 679

osteogenessi imperfecta

Answer 679

an inherited condition affecting collagen maturation and organisation. It is due to a mutation in type I collagen gene . Autosomal dominant inheritance

Question 680

featurs osteogenesisi imperfecta

Answer 680

Low birth weight/length for gestational age, Short stature, 50% scoliosis, Joints: ligamentous laxity resulting in hyperextensible joints, Fractures following minor trauma, Blue sclera, Deafness secondary to otosclerosis, Dental imperfections are common

Question 681

osteogenessi imperfecta

Answer 681

Adjusted calcium, phosphate, parathyroid hormone and ALP results are usually normalin osteogenesis imperfecta

Question 682

mx osteogenesisi imperfecta

Answer 682

physio, surgery, oral calcium, bisphosphonates

Question 683

rickets

Answer 683

a metabolic bone disease characterised by inadequate mineralisation of bone and epiphyseal cartilage in the growing skeleton of children.

Question 684

causes of rickets

Answer 684

Calcium deficiency which is usually due to vitamin D deficiency or nutrition

Question 685

presentation rickets

Answer 685

Metaphyseal swellings, Bowing deformities , Slowing of linear growth, Motor delay, Hypotonia, Fractures, Respiratory distress

Question 686

ix rickets

Answer 686

nvestigations: Bone biopsies: incomplete mineralisation, mildly reduced calcium levels, loss of cortical bone, Imaging: osteopenia – multiple stress factors are common
Biochemical disturbances: Low PO4 (fasting), Serum calcium variable, Raised serum alkaline phosphatase

Question 687

mx rickets

Answer 687

Vitamin D + calcium (possibly IV)

Question 688

neonatal resuscitation

Answer 688

1. Dry the baby, Remove any wet towels and cover , Start the clock or note the time
2. Assess (tone), breathing and heart rate
3. If gasping or not breathing: Open the airway, Give 5 inflation breaths, Consider SpO2 monitoring
4. Re-assess If no increase in heart rate look for chest movement
5. If chest not moving: Recheck head position, Consider 2-person airway control and other airway maneouvres , Repeat inflation breaths, Consider SpO2 monitoring, Look for a response
6. If no increase in heart rate look for chest movement
7. When the chest is moving: If heart rate is not detectable or slow (<60/min), Start chest compressions, 3 compressions to each breath
8. Re-assess heart rate every 30s, If heart rate is not detectable or slow (<60/min), Consider venous access and drugs

Question 689

respiratory distress syndrome

Answer 689

RDS refers to lung disease caused by surfactant deficiency. The disease is largely seen in preterm infants. Rare >32wks gestation. Surfactant is made from around 26 weeks gestation, although adequate levels are not achieved until about 35 weeks. This means that premature babies do not have enough surfactant.

Question 690

who is at increased risk RDS

Answer 690

is associated with: CS delivery, Hypothermia, Perinatal hypoxia, Meconium aspiration, Congenital pneumonia, Maternal diabetes mellitus, Past family history

Question 691

sx RDS

Answer 691

Cyanosis ,Tachypnoea, Chest in drawing, Grunting within 4hr of birth, If untreated, the disease worsens over 48-72hr and then (depending on severity) resolves over 5-7 days

Question 692

ix RDS

Answer 692

Investigations CXR: bilateral, diffuse ‘ground-glass’ appearance (generalised atelectasis), airway bronchograms, reduced lung volume. SpO2 monitoring and blood gases

Question 693

mx RDS

Answer 693

resuscitation.. May need O2, nasal CPAP or ventilation. Surfactant Given as bolus down ETT, penicillin and gentamicin, until congenital pneumonia has been excluded, as it can mimic or coexist with RDS

Question 694

prevention RDS

Answer 694

: Corticosteroids (betamethasone/dexamethasone, 2 doses, 12-hourly) given to mother 1-7 days before birth

Question 695

bronchiopulmonary dysplasia

Answer 695

oxygen requirement at 36/40 corrected gestational age

Question 696

RF bronchiopulmonary dysplasia

Answer 696

Gestational immaturity , Low birth weight, Males , Caucasian heritage, IUGR, Family history of asthma, History of chorioamnionitis

Question 697

sx bronchiopulmonary dysplasia

Answer 697

Rapid breathing, Laboured breathing , Wheezing, The need for continued oxygen therapy after the gestational age of 36 weeks, Difficulty feeding

Question 698

mx bronciopulmonary dysplasia

Answer 698

Oxygen, Monthly RSV injections during RSV season

Question 699

evention bronchipulmonary dysplasia

Answer 699

Caffeine citrate for apnoea of prematurity in infants <1250g, Vitamin A supplementation for infants <1000g

Question 700

sx meconium aspiration

Answer 700

respiratory distress soon after birth. Associated with pulmonary air leaks and PPHN (persistent pulmonary hypertension of the newborn).

Question 701

diagnosis meconium aspiation

Answer 701

CXR – generalised lung over inflation with patchy collapse/ consolidation +/- air leaks.

Question 702

mx meconium aspiration

Answer 702

visualise the larynx and suck out any meconium from larynx/ trachea. supplemental O2, intermittent positive pressure ventilation, surfactant, antibiotics, treat any PPHN.

Question 703

complications mecoinum aspiration

Answer 703

Airway obstruction/collapse, Surfactant dysfunction, Pulmonary vasoconstriction, Chemical pneumonitis, Aspiration pneumonia

Question 704

hypoxic oschaemic encephalopathy

Answer 704

Clinical syndrome of brain injury secondary to a hypoxic-ischaemic insult.

Question 705

causes HIE

Answer 705

Lowered umbilical blood flow e.g. cord prolapse, Low placental gas exchange e.g. placental abruption, Low maternal placental perfusion, Maternal hypoxia from whatever cause, Inadequate postnatal cardiopulmonary circulation

Question 706

presentation HIE

Answer 706

altered: Level of consciousness, Muscle tone , Posture, Tendon reflexes, Suck, Heart rate, CNS homeostasis. Respiratory depression at birth, resuscitation, then encephalopathy within 24hr of birth. Moderate to severe acidosis soon after birth pH <7.0 with a base excess worse than -12.

Question 707

diagnosis HIE

Answer 707

EEG monitoring.

Question 708

mx HIE

Answer 708

Resuscitate at birth, Assess eligibility for therapeutic hypothermia (33-34°C within 6hr of insult, maintained for 72h before gradual rewarming), Monitor and maintain homeostasis, Mild fluid restriction initially as there may be oliguria.

Question 709

neonatal hepatitis syndrome

Answer 709

inflammation of the liver that occurs only in early infancy, usually between one and two months after birth

Question 710

causes neonatal hepatitis syndrome

Answer 710

cytomegalovirus, rubella (measles) andhepatitis A,BorCviruses.

Question 711

sx neoatal hepatitis syndrome

Answer 711

Has Jaundice, failure to thrive, hepatosplenomegaly

Question 712

diagnosiss neonatal hepatits syndrome

Answer 712

viral screen, liver biopsy

Question 713

neonatal hepatitis syndrome

Answer 713

vitamins and supplements, liver transplant

Question 714

apgar score

Answer 714

appearance: 0=blue/pale, 1=pink body blue extremeties, 2=pink
pulse: 0=absent, 1=<100, 2=>100
grimace reflex: 0=floppy, 1=minimal response to stimulation, 2=prompt response to stimulation
activity/muscle tone: 0=absent, 1=flexed arms and legs, 2=active
respiration: 0=absent, 1=slow and irregular, 2=vigorous cry

Question 715

TORCH infection

Answer 715

TORCH infection
T – Toxoplasma gondii
O – Others (Listeria, Varicella, parvovirus B19, Syphilis)
R – Rubella virus
C – Cytomegalovirus (CMV)
H – Herpes simplex virus

Question 716

features congenital toxoplasmosis

Answer 716

Triad: chorioretinitis, hydrocephalus, intracranial calcifications (ring enhancing lesions). Petechiae and purpura

Question 717

mx congenital toxoplasmosis

Answer 717

Pyrimethamine, sulfadiazine and folinic acid. Give spiramycin to fetus in maternal infection

Question 718

prevwention congenital toxoplasmosis

Answer 718

Avoid uncooked meat, cat feces

Question 719

featurs congenital syphilis

Answer 719

Jaundice and hepatosplenomegaly, lymphadenopathy, nasal discharge, maculopapular rash, skeletal abnormalities. Late onset: frontal bossing, Hutchinson triad (saddle nose, Hutchinson teeth, mulberry molars), sensorineural deafness, saber shins

Question 720

mx congenital syphilis

Answer 720

Penicillin

Question 721

prevention congenital syphilis

Answer 721

Penicillin to mother in early pregnancy

Question 722

featurs congenital listeriosis

Answer 722

Spontaneous abortion and premature birth, meningitis, sepsis, vesicular and pustular skin lesions

Question 723

mx congenital listeriosis

Answer 723

Ampicillin and gentamicin

Question 724

prevention congenital listeriosis

Answer 724

Avoid unpasteurised dairy products, avoid cold deli meats

Question 725

features congenital VZV

Answer 725

IUGR, premature, chorioretinitis, cataract, encephalitis, pneumonia, CNS abnormalities, hypoplastic limbs

Question 726

mx cpngenital VZV

Answer 726

VZIG, acyclovir

Question 727

evention congenital VZV

Answer 727

Immunise mother before pregnant, VZIG

Question 728

featurs congenital parvovirus B19

Answer 728

Aplastic anaemia, fetal hydrops

Question 729

mx congenital parvovirus B19

Answer 729

Intrauterine fetal blood transfusion

Question 730

features congenital rubella

Answer 730

Petechiae and purpura. Congenital rubella syndrome: IUGR, sensorineural deafness, cataracts, heart defects, PDA, CNS abnormalities, hepatitis

Question 731

featurs congenital CMV

Answer 731

Jaundice, hepatosplenomegaly, IUGR, chorioretinitis, sensorineural deafness, periventricular calcifications, petechiae and purpura, microcephaly, seizures

Question 732

mx congenital CMV

Answer 732

Ganciclovir and valganciclovir

Question 733

features congenital HSV

Answer 733

Premature, IUGR, skin/eyes and mouth=vesicular lesions, keratoconjunctivits, meningitis, sepsis

Question 734

mx congenital HSV

Answer 734

Acyclovir

Question 735

prevention congenital HSV

Answer 735

CS if lesions

Question 736

physiological jaundice

Answer 736

Appears after 24hr, usually resolves by 14 days. Due to immaturity of hepatic bilirubin conjugation, but poor feeding (particularly in breast-fed infants) can also contribute

Question 737

causes of elevated bilirubin

Answer 737

Exaggerated physiological jaundice e.g. preterm, bruising, Sepsis, Haemolytic disorders, Hepatic disease

Question 738

treatement of elevated biluribin

Answer 738

tart ‘blue light’ phototherapy (converts bilirubin to water-soluble form that can then be excreted in urine) if chart requires, exchange transfusion if very high

Question 739

causes of jaundice in the first 24 hrs

Answer 739

: Assume it is pathological. . Causes: haemolysis, red cell enzyme defects, red cell membrane defects, sepsis, severe bruising.

Question 740

causes of prolonged jaundice

Answer 740

14 days if full term, >21 days if pre-term): Causes: breastfeeding, enclosed bleeding, prematurity, haemolysis, sepsis, hypothyroidism, conjugated jaundice, hepatic enzyme disorders. Investigations: SBR, U&E, FBC, DCT, blood group, thyroid function test, LFTs, glucose

Question 741

causes of conjugated jaundice

Answer 741

sepsis, TPN, biliary tract obstruction (e.g. biliary atresia, choledochal cyst), viral hepatitis, TORCH infections, alpha1-antitrypsin deficiency, cystic fibrosis and others

Question 742

kernicterus

Answer 742

A clinical syndrome resulting from the development of excessive neurotoxic unconjugated bilirubin levels.

Question 743

presentation kernicterus

Answer 743

Lethargy progressing to hypertonia then hypotonia, Poor feeding, Fever, High-pitched cry, Opisthotonos (spasm of the muscles causing backward arching of the head, neck, and spine), Seizures and coma

Question 744

mx kernicterus

Answer 744

intensive care, intensive phototherapy and exchange transfusion, Give IV immunoglobulin

Question 745

complicatios kernicterus

Answer 745

high risk of athetoid cerebral palsy, deafness and low IQ

Question 746

necrotising enterocolitis

Answer 746

bowel of premature infants becomes ischaemic and infected.

Question 747

RF necrotising enterocolitis

Answer 747

Prematurity, IUGR, Hypoxia, Polycythaemia, Exchange transfusion, Hyperosmolar milk feeds

Question 748

presentation necrotising enterocolitis

Answer 748

: Most common in the second week after birth. vomiting (which may be bile streaked) and rectal bleeding (fresh blood in stool), Abdominal distension. Late = Additional abdominal tenderness, Blood, mucus, or tissue in stools, Bowel perforation , Shock , DIC; multi-organ failure.

Question 749

ix necrotising enterocolitis

Answer 749

AXR shows intestinal distension, Pneumatosis intestinalis , Hepatic portal venous gas, Signs of intestinal perforation e.g. free peritoneal gas or gas outlining of falciform ligament (‘football sign’)

Question 750

mx necrotising enterocolitis

Answer 750

Prophylaxis=antenatal steroids and breast milk. Stop milk feeds for 10-14 days. IV broad-spectrum antibiotics for 10-14 days e.g. benzylpenicillin, gentamicin, and metronidazole, parenteral nutrition, may need surgery

Question 751

ause cleft lip and palat

Answer 751

orofacial clefts are due to failure of fusion of maxillary and pre-maxillary processes.

Question 752

presentation cleft lip and palate

Answer 752

: A cleft lip is immediately apparent, A cleft palate will interfere with breastfeeding as it precludes generation of suction.

Question 753

mx cleft lio and palate

Answer 753

Lip repair: at around 3 months of age., Palate repair: at around 6 months of age, Follow-up: long-term because of problems with speech, dentistry, and hearing.

Question 754

gastroschisis

Answer 754

defect in the abdominal wall to the right of the umbilicus. The bowel is eviscerated and not covered by a sac.

Question 755

mx gastroschisis

Answer 755

cover the exposed bowel with Clingfilm, total parenteral nutrition, surgery ASAP-may need to be staged

Question 756

exomphalos

Answer 756

Abdominal contents protrude through abdo wall but covered by amniotic sac
Associated with Down’s syndrome

Question 757

mx exomphalos

Answer 757

CS to reduce risk of rupture, surgery-staged repair

Question 758

sophageal atresia

Answer 758

blind-ending oesophagus. It is more common in trisomies – Downs/Pataus/Edwards

Question 759

resentation oesophageal atresia

Answer 759

: polyhydramnios and a mucousy baby, Excess mucous, Choking and cyanosis on feeding, VACTERL association (Vertebral anomalies (fused vertebrae, hemivertebrae), Anorectal anomalies (imperforate anus), Cardiac anomalies, Trachea-oesophageal fistula, Renal abnormalities, Limb abnormalities (radial ray anomalies, e.g. hypoplastic thumbs))

Question 760

diagnosis oesophageal atresia

Answer 760

Pass a 10F NGT before feeding the baby, CXR: the tube stops in the upper thorax. Air in the stomach indicates a fistula between the trachea and the distal oesophagus

Question 761

mx oesophageal atresia

Answer 761

warm, IV fluids, surgery

Question 762

causes neonatal hypoglycaemia

Answer 762

: Endocrine (Hyperinsulinism, Hypopituitarism, Growth hormone deficiency, Hypothyroidism, Congenital adrenal hyperplasia) Metabolic (Glycogen storage disease, Galactosaemia, Organic acidaemia, Ketonic hypoglycaemia, Carnitine deficiency, Acyl CoA dehydrogenase deficiency) Toxic, Hepatic (Hepatitis, Cirrhosis, Reye syndrome) Systemic (Starvation, Malnutrition, Sepsis, Malabsorption )

Question 763

mx neonatal hyooglycaemia

Answer 763

Asymptomatic child= Oral glucose drink or gel. Symptomatic child=Glucose: 10% 5-10mL/kg, or 25% 2-4mL/kg IV, Followed by: continuous infusion of salt solution with 5-10% glucose (6-8mg/kg/min), e.g. 0.45% saline and 5% glucose.

Question 764

diagnosis neonatal hypoglycaemia

Answer 764

blood glucose value <2.2-2.6mmol/L.

Question 765

definition neonatal early onset sepssi

Answer 765

<72h

Question 766

causes neonatal early onset sepsis

Answer 766

)Group B streptococcus, Listeria, Toxoplasma, Rubella, CMV

Question 767

causes neonatal late onset sepsis

Answer 767

Staphylococcus aureus = commonest, Staph epidermidis, E. coli, Pseudomonas, Klebsiella

Question 768

GBS

Answer 768

Strep agalactiae

Question 769

RF GBS

Answer 769

: Premature birth, Water breaks 18 hours or more before you deliver, Have an infection of the amniotic fluid or placenta, Previous case , Fever during labour

Question 770

sx GBS infection

Answer 770

Fever, Fast, slow or strained breathing, Trouble eating, Extreme fatigue, Irritability, Blue colour to the skin

Question 771

mplications GBS infection

Answer 771

Pneumonia, Meningitis, Sepsis

Question 772

mx GBS infection

Answer 772

IV benzylpenicillin to be given intrapartum for women at risk/ with a positive group B strep swab.

Question 773

sx HSV encephalitis

Answer 773

Focal neurology may or may not be present, Focal or generalised seizures, Depressed level of consciousness

Question 774

mx HSV encephalitis

Answer 774

LP

Question 775

mx HSV encephalitis

Answer 775

acyclovir (IV 10mg/kg, tds for 10-14 days).

Question 776

green traffic light features

Answer 776

Normal colour of skin, lips, and tongue.
Responding normally to social cues. Content andsmiling.
Stays awake or awakens quickly.
Strong normal cry or not crying.
Normal skin turgor and eyes.
Moist mucous membranes.

Question 777

amber traffic light features

Answer 777

Pallor of skin, lips, or tonguereported by parent or carer.
Not responding normally to social cues.
Waking only with prolonged stimulation.
Decreased activity.
Not smiling.
Nasal flaring.
Tachypnoea: 6–12 months of ageRR>50breaths per minute;>12months of ageRR>40breaths per minute.
Oxygen saturation ≤ 95% in air.
Crackles on chest auscultation.
Poor feeding in infants.
Dry mucous membranes.
Capillary refill time of 3seconds or more.
Reduced urine output (in infants ask about wet nappies).
Tachycardia:>160beats/minute under 1year of age;>150beats/minute 1–2years of age;>140beats/minute 2–5years of age.
Fever for 5days or more.
Rigors.
Temperature ≥ 39°C in children 3–6 months of age
Swelling of limb or joint.
Non weight-bearing or not using a limb.

Question 778

red traffic light featues

Answer 778

Pale, mottled, ashen, or blue skin, lips, or tongue.
No response to social cues.
Appears ill to a healthcare professional.
Unable to rouse, or if roused does not stay awake.
Weak, high-pitched, or continuous crying.
Grunting.
Tachypnoea: RR*60breaths per minute or more.
Moderate or severe chest indrawing.
Reduced skin turgor
Temperature ≥ 38°C in infants 0–3 months of age
non-blanching rash
Bulging fontanelle†.
Neck stiffness†.
Focal neurological signs†.
Focal seizures.
Status epilepticus‡.