UK Medical Licensing Assessment (UKMLA) Child Health (Paediatrics and Neonatology) Syllabus

Which vitamin supplements are recommended for breastfed infants in the UK and why?

Vitamin D (all breastfed infants, $8.5$-$10$ mcg/day) because breast milk is low in vitamin D, and vitamin K at birth to prevent haemorrhagic disease of the newborn. Breast milk is also low in vitamin K.

Define delayed and precocious puberty in girls and boys.

Precocious: secondary sexual characteristics before $8$ years (girls) or $9$ years (boys). Delayed: absence by $13$ years (girls) or $14$ years (boys). Delayed puberty is more common and usually benign in boys.

What is the first sign of puberty in girls versus boys, and how is pubertal staging described?

First sign in girls is breast development (thelarche); in boys it is testicular enlargement ($\geq 4$ mL volume). Staged using Tanner stages 1-5.

Define fever in a child and state the highest-risk age group for serious bacterial infection.

Fever is a temperature $\geq 38\,^{\circ}\mathrm{C}$. Infants $<3$ months (especially $<1$ month) are at highest risk; any infant $<3$ months with fever $\geq 38\,^{\circ}\mathrm{C}$ needs full septic screen and admission.

In the NICE traffic light system for the febrile child, give two 'red' (high-risk) features.

Examples: pale/mottled/blue skin, no response to social cues, weak/high-pitched/continuous cry, grunting, tachypnoea $>60$/min, reduced skin turgor, non-blanching rash, bulging fontanelle, age $<3$ months with temp $\geq 38\,^{\circ}\mathrm{C}$, or seizures.

What clinical sign distinguishes viral-induced wheeze/bronchiolitis age groups, and which virus most commonly causes bronchiolitis?

Bronchiolitis typically affects infants $<1$ year (peak 3-6 months) with coryza, cough, wheeze and crackles; viral-induced wheeze affects older preschool children. The commonest cause is respiratory syncytial virus (RSV).

Differentiate croup from epiglottitis by causative organism and key clinical features.

Croup (laryngotracheobronchitis): parainfluenza virus, barking cough, stridor, hoarse voice, usually mild. Epiglottitis: $\textit{Haemophilus influenzae}$ type b, rapid onset, drooling, tripod position, muffled voice, no cough, toxic - do NOT examine the throat.

State the first-line treatment for croup and the key management rule for suspected epiglottitis.

Croup: single dose oral dexamethasone ($0.15$ mg/kg); nebulised adrenaline if severe. Epiglottitis: do not distress the child or examine the throat; secure the airway (senior anaesthetist/ENT) then give IV antibiotics (e.g. cefuroxime).

List the clinical signs used to classify dehydration severity in children, and the fluid deficit percentages.

No clinically detectable dehydration ($<5\%$ loss), clinical dehydration ($5$-$10\%$: dry mucous membranes, reduced urine, tachycardia, reduced skin turgor, sunken eyes), and shock ($>10\%$: decreased consciousness, cold peripheries, prolonged capillary refill, hypotension - a late sign).

What is the formula for calculating the fluid deficit and maintenance fluids using the Holliday-Segar method?

Deficit (mL) $= \%$ dehydration $\times$ weight (kg) $\times 10$. Maintenance: $100$ mL/kg for first $10$ kg, $+50$ mL/kg for next $10$ kg, $+20$ mL/kg for each kg above $20$ kg (per 24 h).

What is the most common cause of gastroenteritis in children, and what is the first-line management of mild dehydration?

Rotavirus (now reduced by vaccination); norovirus also common. First-line for mild dehydration is oral rehydration solution (ORS) given little and often; continue breastfeeding.

What is the resuscitation fluid bolus volume for a shocked child (non-trauma), and what fluid is used?

$10$ mL/kg of an isotonic crystalloid (e.g. $0.9\%$ sodium chloride) given as a rapid bolus, reassess, and repeat as needed. (Up to 20 mL/kg in some protocols; smaller 10 mL/kg boluses in DKA/cardiac/trauma.)

List the components of the paediatric ABCDE assessment of the seriously unwell child.

Airway (with cervical spine control if trauma), Breathing, Circulation, Disability (AVPU/GCS, pupils, glucose - 'Don't Ever Forget Glucose'), Exposure (temperature, rash, full examination).

Classify congenital heart disease into cyanotic and acyanotic, giving examples of each.

Acyanotic (left-to-right shunt or obstruction): VSD, ASD, PDA, coarctation, aortic/pulmonary stenosis. Cyanotic (right-to-left shunt): Tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, truncus arteriosus, TAPVD.

What are the four anatomical features of Tetralogy of Fallot?

(1) Ventricular septal defect, (2) overriding aorta, (3) right ventricular outflow tract obstruction (pulmonary stenosis), and (4) right ventricular hypertrophy. Boot-shaped heart on chest X-ray.

Which duct-dependent lesions require prostaglandin E1 (alprostadil) infusion in the neonate, and why?

Duct-dependent lesions (e.g. transposition of the great arteries, severe coarctation, hypoplastic left heart, pulmonary atresia). Prostaglandin E1 keeps the ductus arteriosus patent to maintain pulmonary or systemic blood flow until surgery.

Describe the inheritance and pathophysiology of cystic fibrosis.

Autosomal recessive mutation in the $\textit{CFTR}$ gene (commonest: $\Delta\mathrm{F508}$) on chromosome 7, encoding a chloride channel. Defective chloride transport produces thick secretions affecting lungs (recurrent infection, bronchiectasis), pancreas (exocrine insufficiency), and gut (meconium ileus).

How is cystic fibrosis diagnosed, and what is the diagnostic sweat test threshold?

Newborn heel-prick screening (raised immunoreactive trypsinogen) confirmed by sweat test and genetic testing. Diagnostic sweat chloride concentration is $>60$ mmol/L.

Compare Down syndrome, Edwards syndrome, and Patau syndrome by chromosome and one key feature each.

Down syndrome - trisomy 21 (hypotonia, single palmar crease, congenital heart disease). Edwards syndrome - trisomy 18 (rocker-bottom feet, overlapping fingers). Patau syndrome - trisomy 13 (cleft lip/palate, polydactyly, microcephaly).

State the karyotypes and one key clinical feature each for Turner syndrome and Klinefelter syndrome.

Turner syndrome: $45,\!X$ (female - short stature, webbed neck, coarctation, primary amenorrhoea/infertility). Klinefelter syndrome: $47,\!XXY$ (male - tall, gynaecomastia, small testes, infertility).

Define a febrile seizure and state the typical age range and key reassuring features of a simple febrile seizure.

A seizure with fever in a child aged $6$ months to $5$ years without CNS infection or metabolic cause. Simple: generalised tonic-clonic, $<15$ minutes, no recurrence within 24 h, complete recovery within 1 hour. Reassure - low risk of epilepsy.

What is the first-line long-term treatment for generalised tonic-clonic seizures and for absence seizures in childhood epilepsy?

Generalised tonic-clonic: sodium valproate (or levetiracetam, especially in girls/women of childbearing potential). Absence seizures: ethosuximide first-line (or sodium valproate). Avoid carbamazepine in absence/myoclonic seizures - can worsen them.

What is the most common childhood cancer, and what blood film/feature suggests it?

Acute lymphoblastic leukaemia (ALL), peak age 2-5 years. Presents with pancytopenia: anaemia (pallor, lethargy), thrombocytopenia (bruising, petechiae), neutropenia (infection), plus bone pain, hepatosplenomegaly and lymphadenopathy; blasts on blood film.

Differentiate nephrotic syndrome from nephritic syndrome in children.

Nephrotic: heavy proteinuria, hypoalbuminaemia, oedema, hyperlipidaemia - commonest cause minimal change disease (steroid-responsive). Nephritic: haematuria, hypertension, oliguria, mild proteinuria - commonest cause post-streptococcal glomerulonephritis.

What is the classic presentation of a Wilms tumour (nephroblastoma)?

A unilateral, painless, palpable abdominal mass in a child under 5 years (often $<3$ years), sometimes with haematuria, hypertension or abdominal pain. It is the commonest renal tumour of childhood.

List clinical features that should raise suspicion of non-accidental injury (NAI) in a child.

Injury inconsistent with the developmental stage or the given history, delayed presentation, changing/inconsistent history, multiple injuries of different ages, certain fracture patterns (metaphyseal, posterior rib, spiral in non-mobile child), bruising in non-mobile infants, retinal haemorrhages, cigarette burns, and torn frenulum.

Define SUDI/SIDS and list the major modifiable risk factors used in safe-sleep advice.

Sudden Unexpected Death in Infancy / Sudden Infant Death Syndrome: sudden unexplained death of an infant $<1$ year. Risk factors: prone or side sleeping, parental smoking, co-sleeping (especially with alcohol/sofa), overheating, prematurity/low birth weight. Advice: 'Back to sleep', feet to foot, smoke-free, avoid overheating.

List the vaccines given at the routine 8-week (2-month) immunisation visit in the UK schedule.

6-in-1 (DTaP/IPV/Hib/HepB), Rotavirus (oral), MenB, and pneumococcal (PCV - from 2024 given at 12 weeks; 6-in-1 + MenB + Rotavirus at 8 weeks).

When are the two doses of the MMR vaccine given in the UK schedule, and what type of vaccine is it?

First dose at $12$ months and second dose at $3$ years $4$ months (pre-school). MMR is a live attenuated vaccine (contraindicated in significant immunosuppression and pregnancy).

Describe the typical age of onset and reassuring management of infantile colic.

Colic: paroxysms of inconsolable crying, often drawing up the legs, in an otherwise well, thriving infant, peaking around 6 weeks and usually resolving by 3-4 months. Management is reassurance and support; it is self-limiting. Use the 'rule of threes' ($>3$ h/day, $>3$ days/week, $>3$ weeks).