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Sickle cell acute chest syndrome — RACP Paediatrics MCQ

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HardHaematology and oncologySickle cell acute chest syndromeRACP Paediatrics

A 9-year-old with sickle cell disease presents with fever, chest pain and increasing oxygen requirement. Chest radiograph shows a new right lower-zone infiltrate and haemoglobin is below baseline. What is the most appropriate management?

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Correct answer: B — Oxygen, broad-spectrum antibiotics and urgent haematology review for transfusion consideration

Fever, chest pain, hypoxia and a new infiltrate define acute chest syndrome, requiring oxygen, antibiotics, analgesia and urgent haematology input, with transfusion considered depending on severity. Treating it as simple pain crisis misses a life-threatening pulmonary complication. The pearl is that acute chest syndrome can evolve during admission for pain, so repeat assessment is essential.

Reference: Therapeutic Guidelines (eTG): Antibiotic; RCH Clinical Practice Guidelines: Sickle cell disease