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Paeds SAQsgastroenterology-hepatology-and-nutrition

Paeds SAQs · gastroenterology-hepatology-and-nutrition

Short-bowel syndrome and intestinal failure — formative SAQs

Two formative SAQs on short-bowel syndrome and intestinal failure in children: a premature neonate after necrotising enterocolitis resection with a high-output jejunostomy testing classification, the sodium principle, IFALD prevention and the rehabilitation ladder; and an older child on home parenteral nutrition testing the role of teduglutide, serial transverse enteroplasty and the indications for transplant.

20 marks30 min1 min readVerification in progress

Target exams

RACP General PaediatricsRACP DWEMRCPCH TheoryABP General Pediatrics
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Study tools

Target exams

RACP General PaediatricsRACP DWEMRCPCH TheoryABP General Pediatrics
Prompt
Short-bowel syndrome and intestinal failure

SAQ 1 — The premature neonate with a high-output jejunostomy (10 marks, 15 minutes)

A baby born at 27 weeks gestation develops necrotising enterocolitis and undergoes a massive small-bowel resection, leaving a 35-centimetre jejunal remnant ending in a jejunostomy with the colon disconnected. On day 14 the stoma output is 60 mL/kg/day, the weight has fallen, and the conjugated bilirubin is rising on parenteral nutrition. [1]

a) Define short-bowel syndrome and intestinal failure, and explain why this child's end-jejunostomy configuration carries a particularly poor prognosis for enteral autonomy. (2 marks) [1]

b) Explain the sodium principle as it applies to this high-output stoma, and state how you would manage the stoma losses acutely. (3 marks) [2]

c) Outline your strategy to prevent intestinal failure-associated liver disease, naming the two dominant modifiable drivers and the lipid approaches used regionally. (3 marks) [5]

d) Describe the staged definitive plan for this child, including the role of enteral feeding, the timing of establishing colon continuity, and the place of teduglutide and transplant. (2 marks) [1] [3]


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References7ShowHide
  1. [1]Duggan CP; Jaksic T Pediatric Intestinal Failure. N Engl J Med, 2017.PMID 28813225
  2. [2]Premkumar MH Nutritional Management of Short Bowel Syndrome. Clin Perinatol, 2022.PMID 35659103
  3. [3]Chiba M; Arnon R; Kori M; et al Efficacy and Safety of Teduglutide in Infants and Children With Short Bowel Syndrome Dependent on Parenteral Support. J Pediatr Gastroenterol Nutr, 2023.PMID 37364133
  4. [5]Lee WS; Teo EH; Chong PY; Poh LK Intestinal failure-associated liver disease (IFALD): insights into pathogenesis and advances in management. Hepatol Int, 2020.PMID 32356227
  5. [8]Dagorno C; Breton A; Lamireau T; et al Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes. J Pediatr Surg, 2025.PMID 39368852
  6. [10]Lee EJ; Iyer KR; Horslen S Pediatric intestinal transplantation. Semin Pediatr Surg, 2022.PMID 35725057
  7. [11]Crenn P; Messing B; Cynober L Citrulline as a biomarker of intestinal failure due to enterocyte mass reduction. Clin Nutr, 2008.PMID 18440672
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