Microvillus inclusion disease: progressive mucosal pathology. A scanning and transmission electron microscopic study, and thoughts about possible pathogenesis.
An infant of African-American descent presented in the immediate newborn period with secretory diarrhea, the cause of which turned out to be microvillus inclusion disease (MID). Small intestinal mucosal biopsies at 6 weeks of age were diagnostic for MID by electron microscopy and repeat biopsies from the small intestine at 15 months demonstrated the seeming relentless progression of this disorder, when a normal structure and organization of small intestinal mucosa was no longer recognizable. Since the child could not tolerate any form of enteral nutrition, a small intestinal transplant was contemplated, but could not be done. The patient did not survive the consequences of an overwhelming sepsis, which resulted in multi-organ failure.
Poley
J R
JR
Section of Pediatric Gastroenterology, Hepatology and Nutrition, Brody School of Medicine, East Carolina University, Greenville, NC 27834, USA. POLEYR@ecu.edu
D001706:Biopsy; D003968:Diarrhea, Infantile / Q000151:congenital / Q000209:etiology / Q000473:pathology; D020895:Enterocytes / Q000648:ultrastructure*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008855:Microscopy, Electron, Scanning / Q000379:methods*; D046529:Microscopy, Electron, Transmission / Q000379:methods*; D008871:Microvilli / Q000648:ultrastructure*; D009081:Mucolipidoses / Q000209:etiology / Q000473:pathology*