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[CD10 expression in a case of microvillous inclusion disease]
Nelly Youssef1, Frank M Ruemmele, Olivier Goulet
1Service d'Anatomie Pathologique, France.
Abstract:
All over the causes of intractable diarrhea of infancy, microvillous inclusion disease is a rare congenital defect of intestinal brush border of unknown aetiology. An autosomal recessive inheritance is suggested by cases occurring in siblings and high incidence of consanguinity. The prognosis of the disease is extremely poor, as life can be sustained only by total parenteral nutrition. Combined bowel-liver or bowel transplantation is regarded as the only potentially life-saving therapy. We report a case of microvillous atrophy who undergone a combined bowel, colonic and liver transplantation, and discuss the tools allowing the light microscopic diagnosis.
Insights
Microvillous inclusion disease is a rare congenital intestinal defect causing intractable diarrhea in infants. This case highlights combined bowel, colonic, and liver transplantation as a potential life-saving therapy for this severe condition.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Pathology
- Surgical Gastroenterology
Background:
- Microvillous inclusion disease (MID) is a rare congenital defect of the intestinal brush border.
- Its etiology remains unknown, though autosomal recessive inheritance is suspected.
- The disease presents with intractable diarrhea in infancy and has a very poor prognosis.
Observation:
- This report details a case of microvillous atrophy, a form of MID.
- The patient underwent a combined bowel, colonic, and liver transplantation.
- Diagnostic tools for light microscopic identification are discussed.
Findings:
- Combined organ transplantation was performed on a patient with microvillous atrophy.
- The study discusses diagnostic methods for identifying this rare condition.
- The prognosis for MID is extremely poor without intervention.
Implications:
- Combined bowel-liver or bowel transplantation offers a potential life-saving treatment for MID.
- Early diagnosis and intervention are crucial for managing this severe congenital disorder.
- Further research into the etiology of MID may lead to novel therapeutic strategies.
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