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.
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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]
References7ShowHide
- [1]Duggan CP; Jaksic T Pediatric Intestinal Failure. N Engl J Med, 2017.PMID 28813225
- [2]Premkumar MH Nutritional Management of Short Bowel Syndrome. Clin Perinatol, 2022.PMID 35659103
- [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
- [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
- [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
- [10]Lee EJ; Iyer KR; Horslen S Pediatric intestinal transplantation. Semin Pediatr Surg, 2022.PMID 35725057
- [11]Crenn P; Messing B; Cynober L Citrulline as a biomarker of intestinal failure due to enterocyte mass reduction. Clin Nutr, 2008.PMID 18440672