Paediatrics👶🏻 Flashcards

1
Q

What are the features of congenital rubella syndrome?

A

Congenital deafness, congenital cataracts, congenital heart disease (PDA and pulmonary stenosis) and learning disability

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2
Q

What are the features of congenital varicella syndrome?

A

Fetal growth restriction, microcephaly, hydrocephalus and learning disability. Scars and significant skin changes in specific dermatomes. Limb hypoplasia and cataracts.

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3
Q

What are the features of congenital cytomegalovirus?

A

Fetal growth restriction, microcephaly, hair loss, vision loss, learning disability, seizures.

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4
Q

What are the features of congenital toxoplasmosis?

A

Intracranial calcification, hydrocephalus, chorioretinitis.

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5
Q

What is the aetiology of Turners Syndrome?

A

XO in which only one sex chromosome is inherited.

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6
Q

Give features of Viral episodic wheeze?

A
  • No interval symptoms
  • No history of excess atopy
  • Likely to improve with age
  • No benefit from regular inhaled steroids
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7
Q

What is epiglottitis caused by?

A

Haemophilus Influenzae

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8
Q

What is the classic cause of croup?

A

Parainfluenza virus (BARKING COUGH)

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9
Q

What is the management of croup?

A

Most cases can be managed at home wth simple supportive treatment.
Oral dexamethasone is very effective. This is usually 150mcg/kg which can be repeated after 12 hours.

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10
Q

How is pneumonia diagnosed?

A

History of cough and/or difficulty breathing (<14 days) with increased respiratory rate
- <2 months - 60/min
- 2-11 - 50/min
- 11 months - 40/min

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11
Q

What is bronchiolitis caused by usually?

A

Respiratory synctial virus is the common pathogen in 78-80%

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12
Q

What are the cyanotic congenital heart diseases?

A
  • Tetralogy of Fallot
  • Transposition of the great arteries
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13
Q

Describe VSD

A

Most common congenital heart defect
S&S: tachycardia, tachyopnea, FTT, HF, pansystolic murmur (L lower stern edge)
Mx: Small – will close spontaneously. Large – surgical closure + diuretics

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14
Q

Describe ASD

A

S&S: commonly none, tachypnea, FTT, wheeze, ejection sytolic murmur (L upper S.E)
Mx: Small – will close spontaneously. Large – surgical closure

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15
Q

Describe PDA

A

DA connects aorta to pulmonary artery
Term infant normally closes by 1/12.
S&S: tachypnea, FTT, continuous machine like murmur (below L clavicle), bounding pulse
Mx: NSAID’s (indomethacin) or surgical ligation

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16
Q

Describe Tetralogy of Fallot

A

4 components?
Pulmonary stenosis, VSD, Overriding aorta, RVH
S&S:
Severe cyanosis, hypercyanotic spells on; exercise, crying, defecating,
squatting on exercise, ejection systolic murmur, clubbing of fingers and toes (late)
Mx:
Surgery at 6 months – close VSD, relieve Pul. outract obstruction

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17
Q

Define transposition of the great arteries

A

Pulmonary artery and Aorta swap
RV –> Aorta –> Around the Body –> RA
LV –> Pulmonary Artery –> Lungs –> LA

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18
Q

Define transposition of the great arteries

A

Pulmonary artery and Aorta swap
RV –> Aorta –> Around the Body –> RA
LV –> Pulmonary Artery –> Lungs –> LA
S&S:
Often present on day 2 of life (after DA closes) with severe life threatening cyanosis
Mx:
Maintain PDA (prostaglandin infusion), Surgical; atrial sepstostomy and correction

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19
Q

What are the signs of a harmless murmur?

A

4 S’s
Soft, Systolic, aSymptomatic, L Sternal edge

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20
Q

What is the presentation of acute epiglottitis?

A
  • Sore throat in a septic looking child
  • Child unable to speak or swallow (drooling)
  • Sitting upright, immobile with open mouth to optimise airway
  • Soft inspiratory stridor, increased resp distress, little/no cough
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21
Q

What is the causative pathogen of whooping cough and what is the incubation period?

A

Bordetella Pertussis
Highly contagious and infectious. Epidemic every 3-4 years
Incubation 10-14 days

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22
Q

What is the management of whooping cough?

A

< 1month Azithromycin 5days
>1 month Azithromycin/ Erythromycin 7days
School exclusion

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23
Q

What is Palivizumab?
How often is it give and who to?

A

Monoclonal antibody
IM once per month through autumn and winter
CF, immunocompromised, congenital heart disease, Downs syndrome

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24
Q

What is a life threatening asthma attack in under 5 ?

A

Sats <92%, silent chest, bradycardic, poor resp effort, altered consciousness, cyanosed

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25
Q

What is a severe asthma attack in over 5 years old?

A

Sats <92%, PEF <50%, unable to complete sentences, HR >125, RR >30 use of accessory neck muscles

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26
Q

What is the gene defect in cystic fibrosis?

A

Autosomal recessive defect in CFTR
Codes cAMP regulated chloride channels in cell membranes (chromosome 7)
Commonest AR in Caucasians, 1 in 25 carriers

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27
Q

How do you diagnose cystic fibrosis?

A
  • Guthrie heel prick screening test
  • Faecal elastase (low levels)
  • Sweat test (Cl ions markedly)
  • Gene abnormalities in CFTR protein
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28
Q

What are the signs and symptoms of pyloric stenosis?

A

Weight loss, FTT, hungry after feeds
Visible gastric peristalsis, palpable abdo mass on feeding

Signs on bloods - Hypokalaemic, hypochloraemic, metabolic alkalosis

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29
Q

What is the management of pyloric stenosis?

A

Ramstedt’s pyloromyotomy

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30
Q

What are the clinical features of intussusception?

A

Severe paroxysmal abdominal colic pain
Child draws knees up to chest, becomes pale, screaming in pain
Vomiting – may become billious
Blood and mucous in stool – REDCURRENT JELLY
RLQ abdo mass – SAUSAGE SHAPED

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31
Q

What signs of intussusception would you see on USS Abdo?

A

Doughnut/target sign

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32
Q

What is the management of intussusception?

A

Rectal air insufflation

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33
Q

How does Intestinal malrotation present?

A

Presents day 1-7 of life:
BILLIOUS VOMITING (below ampulla of vater)
Abdo pain
Tenderness (peritonitis/ischaemic bowel)
Bile stained vomit in 1st week of life = Malrotation until proven otherwise!

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34
Q

What is the management of intestinal malrotation?

A

Ladd’s procedure- rotates bowel anti-clockwise

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35
Q

What is Hirschsprung’s disease?

A

Absence of ganglionic cells from myenteric plexus of large bowel
Results in a narrow, contracted segment of bowel
75% cases confined to recto-sigmoid
Commonly ileum moves into caecum via ileo-caecal valve

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36
Q

What is the triad of findings in haemolytic uraemic syndrome??

A

AKI thrombocytopenia and a normocytic anaemia

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37
Q

When do children receive the MMR vaccine?

A

1 year and 3y4m

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38
Q

When do children receive the HPV vaccine?

A

12-13y

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39
Q

What is the investigation for ALL?

A

Blood film and bone marrow biopsy

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40
Q

How does congenital adrenal crisis present?

A

The symptoms that present and the age at which they become apparent depends on the degree of cortisol and/or aldosterone deficiency.

Boys with severe CAH show symptoms soon after birth, when a baby develops heart rhythm abnormalities, dehydration and vomiting. The levels of minerals (electrolytes) in the body are also affected, particularly showing low salt levels (hyponatraemia) and low blood sugar levels (hypoglycaemia).

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41
Q

What pathogen is involved in Infectious mononucleosis?

A

EBV in 90% of cases. Less frequent causes include CMV and HHV-6

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42
Q

What are ‘sanctuary sights’ in ALL and where are they?

A

The testes and CNS are recognised as areas which are protected from chemotherapeutic agents (due to the existence of the blood brain barrier for CNS.

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43
Q

What treatments are available for sickle cells disease?

A
  • Prophylactic penicillin as the vast majority have had a splenectomy
  • Stem cell transplantation is a curative treatment but is not often done due to the significant risks involved.
  • Hydroxycarbamide is used to prevent vaso-occlusive complications of sickle cell disease.
  • Blood transfusions can be used if the patient is suffering from severe anaemia or to reduce the proportion of Hbs.
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44
Q

What is the deficiency in Haemophilia A?

A

Factor 8

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45
Q

What is the deficiency in Haemophilia B?

A

Factor 9

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46
Q

What are the clinical features of Kawasaki disease?

A

Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands (palmar erythema, swelling)
Burn (fever >5 days)
CRASH and Burn

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47
Q

What do you use to monitor Hodgkin’s lymphoma?

A

Positron emission tomography (PET)

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48
Q

What is the most common age group for Perthes disease?

A

5-10 years

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49
Q

What are some risk factors for developmental dysplasia of the hip?

A

Females are 6x more likely to have DDH
Breech birth, high birth weight, oligohydramnios, prematurity

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50
Q

What is duodenal atresia associated with?

A

Down syndrome

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51
Q

What are some clinical features of children with Down Syndrome?

A

Hypotonia, brushfield spots (increased pigmentation) in the iris, delayed motor milestones, small ears and upslanted palpebral fissures.
Other features include round face, flat occiput, epicanthic folds, protruding tongue, short stature and learning disabilities

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52
Q

What is a common complication of juvenille idiopathic arthritis?

A

Chronic anterior uveitis is seen in up to 1/3 of children with JIA

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53
Q

What are some recognised complications of chickenpox?

A

Bacterial superinfection, cerebellitis, DIC, progressive diseminated disease

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54
Q

What are the clinical features of Fragile X syndrome?

A

learning difficulties, large ears, long thin face, high-arched palate,
marcroorchidism, autism, ADHD hypotonia, mitral valve prolapse

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55
Q

What are the clinical features of Prader-Willi Syndrome?

A

hypotonia, faltering growth, developmental delay, learning difficulties, facies-almond shaped eyes, narrow bridge of nose, narrowing of forehead at temples, thin upper lip.

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56
Q

What are the clinical features of Noonan syndrome?

A

mild learning difficulties, short webbed neck, pectus excavatum, short stature,
congenital heart disease, facies- broad forehead, drooping eyelids, wide distance between eyes

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57
Q

What are the clinical features of Williams syndrome?

A

short stature, congenital heart disease, mild-moderate learning difficulties,
facies- broad forehead, short nose, full cheeks, wide mouth.

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58
Q

What are the clinical features of dermatitis herpetiformis?

A

Itchy, bullous, rash affecting the extensor surfaces (usually) which arise on reddened skin, lesions grow in a centrifugal pattern

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59
Q

What are the clinical features of Impetigo?

A

small pustules that develop a honey-coloured crusted plaques, usually on face, no
surrounding erythema, often not itchy.

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60
Q

What are some risk factors for developmental dysplasia of the hip?

A

Female sex: 6 times greater risk
Breech position
Positive family history
Firstborn children
Oligohydramnios
Birth weight >5kg
Congenital calcaneovalgus foot deformity

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61
Q

Which infants require a routine ultrasound examination for DDH?

A
  • First-degree family history of hip problems in early life
  • Breech presentation at or after 36 weeks gestation, irrespective of presentation at birth or mode of delivery
  • Multiple pregnancy
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62
Q

What is the management of developmental dysplasia of the hip?

A
  • Most unstable hips will spontaneously stabilise by 3-6 weeks of age
  • Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months
  • Older children may require surgery
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63
Q

What is the surgical management of testicular torsion?

A

treatment is with urgent surgical exploration
if a torted testis is identified then both testis should be fixed as the condition of bell clapper testis is often bilateral.

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64
Q

What is Ebstein’s anomaly?

A

Caused by the use of lithium in pregnancy. It occurs when the posterior leaflets of the tricuspid valve are displaced anteriorly towards the apex of the right ventricle.

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65
Q

What is a septic screen?

A

A septic screen is a set of tests and investigations looking for indications of sepsis or infection.

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66
Q

What is the Sepsis Six?

A

Give high flow oxygen
Obtain IV/IO access and take blood tests
Blood gas and lactate (+/- FBC, U&E, CRP if able)
Blood glucose – treat hypoglycaemia (2mls/kg 10% glucose)
Blood cultures
Give IV/IO Antibiotics (dependant on local guidelines)
Consider fluid resuscitation and Monitor urine output
Involve senior clinicians early
Consider inotropic support

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67
Q

What is the most likely pathogen of gram- negative diplococci meningitis?

A

Neisseria Meningitidies

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68
Q

What is the cause of purpura in meningococcal septicaemia?

A

Rash indicates the infection has caused disseminated intravascular coagulopathy and subcutaneous haemorrhages.

The bacteria produce toxins which travel around the body and cause damage to the blood vessels and organs. As the blood vessels become damaged blood leaks into the surrounding tissue.

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69
Q

What is a close contact of meningitis defined as?

A

Risk is highest for people that have had close prolonged contact within the 7 days prior to the onset of the illness

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70
Q

What investigations would you do for JIA?

A

FBC, ESR, CRP, ANA and RF

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71
Q

What initial treatment options would you use in JIA?

A

NSAIDs such as ibuprofen.
Steroids

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72
Q

What is the treatment for Kawasaki Disease?

A

IV Ig
High dose aspirin

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73
Q

What blood tests should you do for new diagnosis T1DM?

A

Baseline bloods (FBC, U&E and a formal lab glucose)
HbA1C
TFT and TPO
Anti-TTG
Insulin antibodies, anti GAD antibodies and islet cell antibodies

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74
Q

What are the most common causes of catastrophic illness in a neonate?

A

Sepsis
Ductal dependent congenital heart disease
Metabolic disturbance

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75
Q

What is stridor?

A

Monophonic, low pitched, turbulent sound that can occur during inspiration or expiration

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76
Q

Which patients might receive palivizumab?

A

Should be used by those at high risk of severe RSV disease
Those with bronchopulmonary dysplasia due to prematurity or chronic lung disease
Those at high risk due to congenital heart disease
Those at high risk due to severe combined immunodeficiency syndrome

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77
Q

What conditions are picked up on newborn screening test?

A

CF
Sickle cell disease
Congenital hypothyroidism
Inherited metabolic disease
Severe combined immunodeficiency

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78
Q

What type of laxative should you avoid in children?

A

Stimulant eg Senna

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79
Q

What are the diagnostic criteria for minimal change disease?

A
  1. Massive proteinuria (3+/4+ on dipstick or urine protein/creatinine ratio >200mg/mmol)
  2. Hypoalbuminemia (<20g/l)
  3. Oedema
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80
Q

When does the moro reflex begin to disappear?

A

Begins to disappear at 12 weeks with complete disappearance by 6 months

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81
Q

What features would you see on examination for a child with osteogenesis imperfecta?

A

Hypermobility, Blue sclera, triangular face, short stature

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82
Q

What are the complications of nephrotic syndrome?

A

Hypercholesterolaemia, Thrombosis, risk of infection, hypovolaemia.

83
Q

What is the prognosis of children with nephrotic syndrome?

A

1/3 with steroid-sensitive nephrotic syndrome experience frequent relapses, 1/3 experience infrequent relapses and 1/3 experiences no relapses at all

84
Q

What is the most common nephrotic syndrome in children?

A

Minimal change disease

85
Q

What surgery may be on offer in severe GORD?

A

Nissen Fundoplication

86
Q

What clinical signs would you see in acute otitis media?

A

Red, bulging and tender tympanic membrane

87
Q

What sign would you see in otitis media with effusion?

A

You would see a grey tympanic membrane, loss of cone reflex, visible fluid level behind the tympanic membrane.

88
Q

What is a naevus flammeus caused by and what is another name for this condition?

A

=Port-wine stain
Caused by a vascular malformation of the capillaries in the dermis

89
Q

How do you diagnose Hirschprung’s disease?

A

Suction rectal biopsy

90
Q

Name some complications of GORD?

A

Recurrent pulmonary aspiration and Sandifer syndrome

91
Q

What is the first line treatment for a patient under 2 years of age with bilateral acute otitis media?

A

5 day course of amoxicillin
Clarithromycin is a second line antibiotic if patient is allergic to penicillin

92
Q

If a rectal biopsy shows absence of ganglion cells in the mesenteric plexus what condition does this indicate?

A

Hirschsprung’s disease

93
Q

What is the name of the procedure used to treat Hirschsprung’s disease?

A

Swenson procedure. This removes the section of affected bowel and anastomoses the remaining sections of bowel together.

94
Q

What murmur would you hear with ventricular septal defects?

A

Pansystolic murder heard loudest at the lower left sternal edge

95
Q

What murmur would you hear with patent ductus arteriosus?

A

Continuous machinery murmur typically heard at the upper-left sternal border

96
Q

What murmur would you hear with atrial septal defects?

A

Ejection systolic murmur heard loudest at the upper-left sternal border

97
Q

What is the surgical procedure used to treat volvulus?

A

Ladd’s procedure

98
Q

What is the name of the procedure used to manage cases of biliary atresia?

A

Kasai procedure

99
Q

What is the inheritance of achondroplasia?

A

It is an autosomal dominant disorder associated with short stature. In most cases (70%) it occurs as a sporadic mutation.

100
Q

What are the clinical features of achondroplasia?

A
  • Short limbs (rhizomelia) with shortened fingers (brachydactyly)
  • Large head with frontal bossing and narrow foramen magnum
  • Midface hypoplasia with a flattened nasal bridge
  • ‘Trident’ hands
  • Lumbar lordosis
101
Q

In an infant (under 1 year) what pulses would you check in BLS?

A

Carotid and femoral pulse

102
Q

How long do you wait in stable umbilical hernias in children?

A

Usually no treatment is required as they typically resolve by 3 years of age. If not they are usually managed ages 4-5 with an elective outpatient surgical repair.

103
Q

At what centile for height should children be reviewed by a paediatrician?

A

Below 0.4th centile

104
Q

What is the management for mild/moderate seborrhoeic dermatitis?

A

Baby shampoo and baby oils

105
Q

If a child’s asthma is not completely managed by SABA+ low dose ICS what do you do next?

A

Add in Leukotriene receptor antagonist (LTRA)

106
Q

What is the most common cause of ambiguous genitalia in newborns?

A

Congenital adrenal hyperplasia

107
Q

What are some features suggestive of hypernatraemic dehydration?

A
  • Jittery movements
  • Increased muscle tone
  • Hyperreflexia
  • Convulsions
  • Drowsiness or coma
108
Q

What is the most common cause of death in Measles?

A

Pneumonia

109
Q

What is the investigation of choice for reflux nephropathy?

A

Micturating cystography
A DMSA scan may also be performed to look for renal scarring

110
Q

What is Gastroschisis?

A

A congenital defect in the anterior abdominal wall just lateral to the umbilical cord

111
Q

What is the management for gastroschisis?

A
  • Vaginal delivery may be attempted
  • Newborns should go to theatre as soon as possible after delivery e.g. within 4 hours
112
Q

What is exomphalos?

A

The abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum

113
Q

What is the management for exomphalos?

A
  • Caesarean section is indicated to reduce the risk of sac rupture
  • A staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure
114
Q

What the most common example of a Robertsonian translocation?

A

Down syndrome

115
Q

What is DiGeorge syndrome a deletion of and what signs should this result in?

A

Due to deletion at 22q11
Widely varying clinical effects resulting in congenital defects typically cleft lip and palate, congenital heart problems and sometimes also learning difficulties. There may also be a weakened immune response

116
Q

What is William’s Syndrome a deletion of and what clinical features do you get?

A

7q11
May result in learning difficulties, although individuals are often cheerful and very friendly, especially to strangers. May have abnormal facial appearance

117
Q

What is Cri du chat syndrome a deletion of and what are the clinical features?>

A

5p

118
Q

What are the resuscitation fluids used in DKA?

A

Initial 20ml/kg of 0.9% sodium chloride as a bolus (given over 15mins)

119
Q

What changes occur to the fetal circulation within minutes of birth?

A
  • Constriction of the umbilical vessels
  • Constriction of the ductus venosus
  • Constriction of the ductus arteriosus
  • Closure of the foramen ovale
120
Q

What changes occur to the fetal circulation within minutes of birth?

A
  • Constriction of the umbilical vessels
  • Constriction of the ductus venosus
  • Constriction of the ductus arteriosus
  • Closure of the foramen ovale
121
Q

What is the most common cause of pulmonary hypoplasia?

A

Congenital diaphragmatic hernia

122
Q

When can a child with scarlet fever return to school?

A

24 hours after commencing antibiotics

123
Q

What does minimal change look like on histology?

A

Minimal or no histological changes on biopsy

124
Q

What is the most common small vessel vasculitis?

A

Henoch-Schonlein Purpura which most commonly affects children aged 3-5 years old

125
Q

What is the management of HSP?

A

-NSAIDs for analgesia and their anti-inflammatory effect
-Antihypertensives may be needed to control blood pressure
-After an episode of HSP, children should have regular urine dips for 12 months to check for renal impairment.

126
Q

What is the management of HSP?

A

-NSAIDs for analgesia and their anti-inflammatory effect
-Antihypertensives may be needed to control blood pressure
-After an episode of HSP, children should have regular urine dips for 12 months to check for renal impairment.

127
Q

What do you use for tonsillitis if allergic to penicillin?

A

Erythromycin or clarithromycin

128
Q

What is the pathogen responsible for roseola?

A

Human herpes virus 6

129
Q

What is the management of roseola?

A

Supportive

130
Q

How does Ewing’s sarcoma present?

A

It most commonly affects teenages and young adults with the pelvis, thigh bone and shin bone being the most commonly affected areas.
- Bone pain particularly occuring at night
- A mass or swelling
- Restricted movement in a joint

131
Q

How many episodes in a year of tonsillitis do you need to have for tonsillectomy?

A

Seven or more in a year,
Five or more/year in two years
Three or more episodes/year in three years

132
Q

What happens in a vaso-occlusive crises?

A

These are a common, painful complication of sickle cell disease.
The sickled red blood cells clump together and occlude vessels, resulting in ischaemia of downstream tissues.

133
Q

What is the Potter sequence?

A

Potter’s sequence describes the typical physical appearance caused by pressure in utero due to oligohydramnios. When it is a result of bilateral renal agenesis, it it called potter syndrome

134
Q

What are the facial signs of Potter syndrome?

A
  • Flattened ‘parrot-beaked’ nose
  • Recessed chin
  • Downward epicanthal folds
  • Low-set, cartilage-deficient eats (known as Potter’s ears)
135
Q

What do you do if you find an intermittent squint at the NIPE?

A

Refer in still present at 8 weeks of life

136
Q

What is the Paul Bunnell test used for?

A

It is positive in EBV infection

137
Q

What does the Guthrie test screen for?

A
  • Hypothyroidism
  • Phenylketonuria
  • Galactosaemia
  • Maple syrup urine disease
  • Homocystinuria
138
Q

What is the second line treatment for ADHD in school aged children?

A

Lisdexamfetamine

139
Q

What are the DSM-V 6 criteria for ADHD?

A
  • Five or more symptoms of inattention and/or ≥5 symptoms of hyperactivity/impulsivity must have persisted for ≥6 months to a degree that is inconsistent with the developmental level and negatively impacts social and academic/occupational activities.
  • Several symptoms (inattentive or hyperactive/impulsive) were present before the age of 12 years.
  • Several symptoms (inattentive or hyperactive/impulsive) must be present in ≥2 settings (eg, at home, school, or work; with friends or relatives; in other activities).
  • There is clear evidence that the symptoms interfere with or reduce the quality of social, academic, or occupational functioning.
  • Symptoms do not occur exclusively during the course of schizophrenia or another psychotic disorder, and are not better explained by another mental disorder (eg, mood disorder, anxiety disorder, dissociative disorder, personality disorder, substance intoxication, or withdrawal).
140
Q

What is the first line treatment for VIW?

A

Inhaled Salbutamol

141
Q

What are the common signs and symptoms for NEC?

A
  • Abdominal distension
  • Vomiting
  • Visible intestine loops lacking peristalsis
  • Rectal bleeding
  • Lethargy
  • Feeding intolerance
142
Q

What would you see on X-Ray for NEC?

A
  • Dilated bowel loops
  • Bowel wall oedema
  • Pneumotitis Intestinalis
143
Q

What congenital heart defect may be seen in William’s Syndrome?

A

Supravalvular aortic stenosis

144
Q

What is the treatment for a SEVERE exacerbation of asthma?

A

High flow O2 (if SpO2<94%) Corticosteroids, Nebulised salbutamol and nebulised ipratropium

145
Q

What are the features of the rash seen in meningococcal septicaemia?

A

Non-blanching purpuric flat rash.
It may start anywhere on the body

146
Q

What are the clinical features of intusussception?

A
  • Paroxysmal episodes of colicky abdominal pain
  • Features of intestinal obstruction
  • As it is causing obstruction there is distension of the abdomen
147
Q

What is Scarlet fever caused by?

A

Group A Streptococci
Usually Streptococcus pyogenes

148
Q

What are some of the features of growing pains?

A
  • Never present at the start of the day after the child has woken
  • No limp or limitation of physical activity
  • Systemically well
  • Normal physical examination
  • Motor milestones normal
  • Symptoms are often intermittent and worse after a day of vigorous activity
149
Q

What is the stepwise pathway for Asthmatic children under 5 years of age?

A

1) SABA
2) SABA + 8 week trial of paediatric moderate dose ICS (if symptoms resolve change to low dose ICS)
3) SABA + paediatric low-dose ICS + Leukotriene receptor antagonist (LTRA)
4) Stop the LTRA and refer to a paediatric asthma specialist

150
Q

What is the paediatric low dose of ICS?

A

<= 200 microgrames or budesonide or equivalent

151
Q

What is the paediatric moderate dose of ICS?

A

200-400 micrograms budesonide or equivalent

152
Q

What is the paediatric high dose of ICS?

A

> 400 micrograms budesonide or equivalent

153
Q

What is the paediatric high dose of ICS?

A

> 400 micrograms budesonide or equivalent

154
Q

What is the defect caused in Ebstein’s anomaly?

A
  • Tricuspid regurgitation (pan-systolic murmur)
  • Tricuspid stenosis (mid-diastolic murmur)
  • Also enlargement of the right atrium
155
Q

If there is bilateral testes enlargement in precocious puberty what might this indicate as a cause?

A

Gonadotrophin release from intracranial lesion

156
Q

If there is unilateral testes enlargement in precocious puberty what might this indicate as a cause?

A

Gonadal tumour

157
Q

If there are small testes in precocious puberty what might this indicate as a cause?

A

Adrenal cause either a tumour or adrenal hyperplasia

158
Q

What figure of blood glucose is commonly used for neonatal hypoglycaemia?

A

<2.6mmol/L§

159
Q

What may persistent/severe hypoglycaemia be caused by?

A
  • preterm birth (< 37 weeks)
  • maternal diabetes mellitus
  • IUGR
  • hypothermia
  • neonatal sepsis
  • inborn errors of metabolism
  • nesidioblastosis
  • Beckwith-Wiedemann syndrome
160
Q

What are the autonomic features of neonatal hypoglycaemia?

A

‘Jitteriness”, Irritable, tachypnoea, pallor

161
Q

What are the neuroglycopenic features of neonatal hypoglycaemia?

A

Poor feeding/sucking, weak cry, drowsy, hypotonia, seizures

162
Q

If neonatal hypoglycaemia is asymptomatic how do you manage this?

A

Encourage normal feeding (either bottle or breast)
Monitor blood glucose

163
Q

If neonatal hypoglycaemia is symptomatic or very low blood glucose how do you manage this?

A

Admit to the neonatal unit
Intravenous infusion of 10% dextrose

164
Q

What is the definitive management of a slipped capital femoral epiphysis?

A

Refer to orthopaedics for in situ fixation with a cannulated screw

165
Q

What is the most common cause of pulmonary hypoplasia?

A

Congenital diaphragmatic hernia

166
Q

What are some risk factors for atopic eczema?

A

Food allergies. Asthma. FH of atopic diseases

167
Q

How does a varicocele usually present?

A

Usually asymptomatic but some patients may present with a dull aches.

168
Q

What is a varicocele?

A

It is a scrotal swelling which occurs due to dilated testicular veins. They occur in 15% of boys usually around puberty and in the left testicle.

169
Q

What are some risk factors for Cerebral Palsy?

A

Low birth weight, Birth complications (placental abruption etc), Maternal infections, maternal thyroid dysfunction, prematurity.

170
Q

How does maternal infection increase risk of cerebral palsy?

A

Results in cytokine release which can cause inflammation and damage of the brain.

171
Q

What do you do with a child with croup who does not respond to oral dexamethasone?

A

Inhaled oxygen and nebulised adrenaline

172
Q

What are the four key drivers for failure to thrive?

A

1) Inadequate intake
2) Inadequate retention
3) Malabsorption - Such as coeliac disease
4) Increased requirements

173
Q

Where are foreign objects most likely to be found if aspirated?

A

Right main bronchus

174
Q

What would a child with ASD present like?

A

Avoids eye contact, change to regime causes stress and stereotypical repetitive movements

175
Q

What drugs do you give for bacterial meningitis in a 4-month old?

A

IV Ceftriaxone and dexamethasone

176
Q

What drugs do you give for bacterial meningitis in a 3 month old?

A

IV Cefotaxime and Amoxicillin

177
Q

What is the first line choice of antibiotic for suspected early-onset infection in a neonate?

A

Use intravenous benzylpenicillin with gentamicin
Benzylpenicillin (dosage of 25mg/kg every 12 hours)
Gentamicin (5mg/kg every 36 hours)

178
Q

If babies have a positive blood culture, how long should you give antibiotics for in neonatal early onset infection?

A

7 days

179
Q

What are the major causes of non-billious vomiting in infants?

A
  • Pyloric stenosis
  • GORD
  • Infection (especially UTI and gastroenteritis)
180
Q

What is the first-line investigation for pyloric stenosis?

A

Ultrasound scan if a mass is not clinically palpable which is around 99% sensitive and specific.
Upper GI contrast XR is an alternative

181
Q

Examples of autosomal dominant inherited conditions?

A
  • Polycystic kidney disease
  • Familial hypercholesterolaemia
  • Marfan’s Syndrome
  • Huntington’s disease
  • BRCA1/BRCA2
182
Q

Examples of autosomal recessive conditions?

A
  • Phenylketonuria
  • Haemochromatosis
  • Spinal muscular atrophy
  • Non-syndromic sensorineural deafness
183
Q

Examples of X-Linked conditions?

A
  • Duchenne and Becker Muscular dystrophy
  • Haemophilia
  • Fragile X syndrome
  • Retinitis Pigmentosa
  • Ocular Albinism
184
Q

Examples of mitochondrial disorders?

A
  • MELAS: Mitochondrial encephalopathy, lactic acidosis, stroke like episodes
  • MERRF: Myoclonic epilepsy, ragged red fibres
  • Mitochondrially inherited DM and deafness
  • Leber’s hereditary optic atrophy
185
Q

Examples of genomic imprinting?

A

Prader-Willi Syndrome (caused by deletion of paternally inherited chromosome 15 or maternal uniparental disomy)
Angelman’s syndrome
(caused by a deletion in the maternally inherited chromosome 15 or paternal uniparental disomy)

186
Q

Till what age should children be measured lying down?

A

2 years of age

187
Q

When is Newborn blood spot screening test repeated?

A

In babies <32 weeks the sample is repeated at day 28 of life. The test should be carried out between day 5 and 8 regardless of medical condition, milk feeding and prematurity.

188
Q

When can regular cow’s milk be introduced into a child’s diet?

A

Regular cow’s milk should not be introduced before the age of 12 months as it can cause microscopic bleeding leading to iron deficiency.

189
Q

What is the pathophysiology in bronchiolitis?

A
  • Proliferation of goblet cells causing excess mucus production
  • IgE reaction causing inflammation
  • Bronchial and bronchiolar constriction - Submucosal oedema
190
Q

What drug can be given for severe cases of bronchiolitis?

A

Ribavirin

191
Q

What is the pathophysiology of Asthma?

A

Driven by Th2 T cells, allergens arrive at the airway in dendritic cells and cause an exaggerated immune response
- Increased mast cells and eosinophils
- Bronchoconstriction and exudate release

192
Q

What is Congenital adrenal hyperplasia a deficiency of?

A

21-Hydroxylase

193
Q

What is the Gold standard test for congenital adrenal hyperplasia?

A

Raised serum 17a-hydroxyprogesterone

194
Q

When are babies born to HIV positive mothers tested?

A

HIV viral load test at 3 months
HIV antibody test at 24 months

195
Q

What is the inheritance of Von Willebrand’s disease and where is the mutation?

A

Autosomal dominant condition due to mutation of the vWF gene

Deficiency of vWF –> Defective platelet plug

196
Q

What is the management for Von Willebrand’s disease?

A

Desmopressin
Severe = Plasma derived FVIII concentrate

197
Q

What is the pathophysiology of Sickle Cell anaemia?

A

It is a point mutation of the B-Globin gene –> Valine swapped for glutamine

198
Q

What is a craniopharyngioma?

A

A developmental tumour arising from the squamous remnant of Rathke pouch

199
Q

What is the karyotype of Klinefelter syndrome?

A

47, XXY

200
Q

What is the Sillence classification of osteogenesis imperfecta?

A

● I – mildest
○ Autosomal dominant
○ Blue sclera
○ Presents at preschool age. Hearing deficit in 50%

● II – lethal
○ Autosomal recessive
○ Blue sclera
● III – most severe survivable form
○ Autosomal recessive
○ Normal sclera
○ Fractures at birth, progressively short stature
● IV – moderate severity
○ Autosomal dominant
○ Normal sclera
○ Bowing bones and vertebral fractures, normal hearing.

201
Q

What is the commonest cause of AKI in children?

A

Haemolytic uraemic syndrome

202
Q

What is osteomyelitis commonly due to ?

A

Staph Aureus

203
Q

What does RDS look like on CXR?

A

Ground glass appearance