NRE Step 1 Paediatrics MCQs

Answer: No oxygen; NG feeds, admit. Supportive care: feeding support when intake falls below about 50–75%, oxygen if SpO₂ below 90% (over 6 weeks).

• Discharge: Feeding at 50% needs support.
• Nebulised salbutamol: Doesn't help.
• Oral antibiotics: Viral illness.
• Oxygen to 98%: Oxygen is needed below 90% in this age.

Answer: Knee–chest position + O₂. Tetralogy of Fallot consists of a VSD, overriding aorta, right ventricular outflow obstruction and right ventricular hypertrophy. Hypercyanotic ("tet") spells occur when outflow obstruction worsens and blood shunts right to left. Knee–chest position (like squatting) increases systemic vascular resistance and pulmonary flow. Give oxygen, morphine, fluids and, if needed, beta-blockers or phenylephrine. Surgery repairs the defect.

• Lie flat, IV furosemide: Diuretics reduce preload and worsen the spell.
• IV adenosine: Adenosine treats SVT; a tet spell is a sudden rise in right-to-left shunting and needs knee–chest positioning and oxygen.
• Encourage crying to raise oxygen: Crying increases pulmonary resistance and right-to-left shunting, making the spell worse; the child should be calmed.

Answer: Oral antibiotic (e.g. azithromycin) + ORS. Blood in the stool in a child (dysentery) is usually due to Shigella; WHO recommends an oral antibiotic effective locally (ciprofloxacin per WHO; azithromycin where resistance is high) for 3 days, plus fluid management, zinc and continued feeding, with follow-up in 2 days. Antimotility drugs are contraindicated. Consider amoebiasis if no response and trophozoites with ingested red cells are seen.

• ORS and zinc only, without antibiotics: Bloody diarrhoea with fever (dysentery) is usually Shigella and needs an antibiotic as well as ORS and zinc.
• Loperamide with ORS and zinc: Loperamide is contraindicated in children and in bloody diarrhoea.
• Oral metronidazole as first-line: Metronidazole is used only when amoebic dysentery is confirmed; Shigella is far more common.

Answer: Kasai portoenterostomy. Prolonged jaundice (> 14 days term, > 21 days preterm) needs a split bilirubin. Conjugated hyperbilirubinaemia with pale stools and dark urine suggests biliary atresia — progressive obliteration of extrahepatic ducts. Early Kasai portoenterostomy (ideally < 45–60 days) improves outcome; many still need liver transplantation. Stool colour cards aid early detection.

• Intensive phototherapy: Phototherapy is for unconjugated jaundice.
• Stop breastfeeding for 48 h: This is not breast milk jaundice.
• Ursodeoxycholic acid alone: Ursodeoxycholic acid is not definitive.

Answer: Enuresis alarm. Primary nocturnal enuresis is common (15% at 5 years, resolving ~15%/year). Under 5 — reassurance. From 5–7 years: reassure, regular daytime fluids, toileting before bed, star charts for agreed behaviours (not dry nights), no punishment. Enuresis alarm is first-line with best long-term success; desmopressin for rapid short-term control (camps/sleepovers) or if alarm unsuitable — avoid fluids 1 h before and 8 h after (hyponatraemia). Investigate secondary enuresis (UTI, diabetes, constipation, stress, abuse).

• Desmopressin forever: Desmopressin works short term, but relapse is common when it is stopped.
• Fluid restriction all day: Restricting fluids all day is not advised.
• Punishment for wet nights: Punishment is harmful and ineffective.

Answer: Hyperdiploidy or t(12;21). ALL is the commonest childhood cancer (peak 2–5 years). Good prognosis: age 1–10, WBC < 50 × 10⁹/L, B-cell lineage, hyperdiploidy, t(12;21) ETV6–RUNX1, rapid response (MRD negative). Poor: infants < 1 or > 10 years, WBC > 50, T-cell, CNS disease, t(9;22), KMT2A rearrangements, hypodiploidy. Overall cure ~90% with risk-adapted chemotherapy including CNS prophylaxis.

• t(9;22) Philadelphia: The Philadelphia translocation carries a poor prognosis.
• KMT2A (MLL) rearrangement: KMT2A rearrangement carries a poor prognosis, often in infants.
• Hypodiploidy: Hypodiploidy carries a poor prognosis.

Answer: HbA2 by HPLC. β-thalassaemia trait: microcytosis (MCV < 75), normal/raised RBC count, normal ferritin, mild or no anaemia; HbA2 > 3.5% on HPLC/electrophoresis confirms. If both partners are carriers, each pregnancy has a 25% chance of thalassaemia major — offer genetic counselling and prenatal diagnosis (CVS at 10–12 weeks). Pakistan’s provincial laws (e.g. Punjab Thalassaemia Prevention Act) promote premarital and family screening.

• Serum iron and TIBC: Iron studies do not confirm thalassaemia trait.
• Bone marrow aspirate: Bone marrow is not needed.
• Reticulocyte count: Reticulocyte count is not diagnostic.

Answer: Hospital admission for medical stabilisation. Anorexia nervosa: restriction of intake, intense fear of weight gain, disturbed body image. Medical instability (HR < 50 day, BP < 80/45, postural changes, temperature < 35.5, electrolyte abnormalities, syncope, BMI very low, arrhythmias) requires admission for cautious refeeding (monitor phosphate, K, Mg — refeeding syndrome) and cardiac monitoring. Family-based therapy is first-line for adolescents.

• Outpatient dietitian advice only: She is medically unstable.
• Start high-calorie feeding rapidly at home: Rapid feeding at home risks refeeding syndrome.
• SSRIs alone: SSRIs are not first-line in anorexia.

Answer: 25 per 1000 live births. Neonatal mortality rate = deaths in first 28 days ÷ live births × 1000 = 300/12,000 × 1000 = 25. Pakistan’s NMR is among the highest in the world (~40/1000), driven by prematurity, birth asphyxia and sepsis. Infant mortality rate: deaths < 1 year per 1000 live births. Under-5 mortality: deaths < 5 years per 1000 live births. Perinatal mortality: stillbirths (≥ 28 weeks) + early neonatal deaths (first 7 days) per 1000 total births.

• 2.5 per 1000 live births: 2.5 is a decimal error; 300 ÷ 12,000 × 1,000 = 25.
• 25 per 100,000 live births: The denominator is per 1,000, not 100,000.
• 250 per 1000 live births: 250 is an arithmetic error.

Answer: Trisomy 18. Edwards syndrome (trisomy 18): clenched hands with overlapping fingers, rocker-bottom feet, micrognathia, prominent occiput, low-set ears, cardiac defects; most die in the first year. Patau (trisomy 13): holoprosencephaly, cleft lip/palate, polydactyly, microphthalmia, scalp defects (cutis aplasia). Down (21): hypotonia, upslanting palpebral fissures, single palmar crease, AVSD, duodenal atresia.

• Trisomy 13: Trisomy 13 causes cleft lip/palate and polydactyly.
• Trisomy 21: Trisomy 21 has different features.
• 45,X: 45,X affects females with lymphoedema.

Answer: Mebendazole for all household. Enterobius vermicularis (threadworm/pinworm): nocturnal perianal itch as females lay eggs; sticky tape (Scotch tape) test in the morning. Treat all household members with mebendazole (or albendazole/pyrantel) single dose, repeat after 2 weeks; strict hygiene (handwashing, short nails, morning baths, washing bedding) for 6 weeks. Ascaris: intestinal obstruction, Loeffler pneumonitis; hookworm: iron-deficiency anaemia.

• Metronidazole for 5 days: Metronidazole treats Giardia or amoebiasis.
• Single-dose praziquantel: Praziquantel treats tapeworms or schistosomes.
• Hygiene measures alone: Hygiene alone is not enough; treat with a drug as well.

Answer: Persistent pulmonary hypertension. PPHN: failure of normal fall in pulmonary vascular resistance after birth → right-to-left shunting via PDA/PFO → hypoxaemia with pre-/post-ductal saturation difference (> 3–5%). Causes: meconium aspiration, sepsis, asphyxia, diaphragmatic hernia, maternal SSRIs/NSAIDs. Management: oxygen, ventilation, surfactant, inhaled nitric oxide, sedation, avoid acidosis/hypothermia; ECMO.

• Transposition of great arteries: In TGA the heart is structurally abnormal.
• Respiratory distress syndrome: RDS occurs in preterm babies with surfactant deficiency.
• Transient tachypnoea of newborn: TTN is mild and resolves.