CF – pancreatic insufficiency — RACP Paediatrics MCQ

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EasyRespiratoryCF – pancreatic insufficiencyRACP Paediatrics

A child newly diagnosed with cystic fibrosis has steatorrhoea and failure to thrive despite adequate caloric intake. Faecal elastase is low. What is the most important specific treatment to initiate?

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Correct answer: E — Pancreatic enzyme replacement therapy

Explanation lettering: C = shown as A · E = shown as B · D = shown as C · B = shown as D · A = shown as E

Pancreatic exocrine insufficiency occurs in ~85% of CF patients. Pancreatic enzyme replacement therapy (PERT) taken with all meals and snacks is essential to treat malabsorption. Dosing is adjusted based on weight, fat intake, and stool output. Fat-soluble vitamin supplementation (A, D, E, K) is also required.

Reference: RCH Melbourne – 2023 – Cystic Fibrosis CPG; Australian Therapeutic Guidelines – 2024