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Cyclosporin-responsive enteropathy and protracted diarrhea
M Coulthard1, J Searle, M Patrick
1Department of Gastroenterology and Nutrition, Royal Children's Hospital, Brisbane, Australia.
Insights
A child with severe secretory diarrhea and intestinal inflammation refractory to standard treatments experienced dramatic recovery with cyclosporin. This suggests immune factors play a role and cyclosporin may be a valuable treatment option.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Inflammatory Bowel Disease
Background:
- Severe protracted secretory diarrhea and subtotal villus atrophy can be debilitating in children.
- Refractory cases often lack identifiable infectious or metabolic causes, necessitating extensive supportive care.
Observation:
- A 19-month-old child presented with severe secretory diarrhea, subtotal villus atrophy, and intestinal inflammation.
- The condition was refractory to nutritional support, steroids, and anti-inflammatory agents, requiring 18 months of total parenteral nutrition.
Findings:
- Introduction of cyclosporin led to a dramatic resolution of secretory diarrhea and enteropathy.
- Discontinuation of cyclosporin resulted in symptom relapse, with improvement upon reintroduction.
Implications:
- This case highlights a potential immune-mediated mechanism in refractory pediatric enteropathy.
- Cyclosporin demonstrates therapeutic potential for similar cases, warranting further investigation.
Abstract:
We describe a child born to unrelated parents who developed severe protracted secretory type diarrhea associated with subtotal villus atrophy and intestinal inflammation at the age of 19 months. No infectious, metabolic, or anatomical basis for this condition was identified and the child required total parenteral nutrition for a period of 18 months despite trials of special enteral formulas, steroids, and anti-inflammatory agents. This refractory "enteropathy" responded dramatically to the introduction of cyclosporin, with cessation of the secretory diarrhea, recovery from the enteropathy, and cessation of parenteral nutrition. The symptoms relapsed when cyclosporin was briefly discontinued and improved following reintroduction of this drug. This experience suggests a role for immune factors in the pathogenesis of the enteropathy in this case and that a trial of cyclosporin is worthy of consideration in similar cases.