Intestinal Microvillous Atrophy in a Patient with Down Syndrome and Aganglionic Megacolon

Intestinal microvillous disorders are an uncommon cause of severe diarrhea, with very poor prognosis. The authors report the case of a female infant with Down syndrome, aganglionic megacolon, severe diarrhea, and jejunal biopsy with ultrastructural changes consistent with microvillous atrophy. The p...

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Bibliographic Details
Published in:Ultrastructural pathology 2002, Vol.26 (1), p.41-45
Main Authors: Martínez, Miguel A., Egea, Anastasio Serrano, López, Javier Manzanares, Benítez, Enrique Medina
Format: Article
Language:English
Subjects:
Acetylcholinesterase - analysis
Atrophy - complications
Atrophy - pathology
Colon - enzymology
Colon - pathology
Colostomy
Diarrhea, Infantile - etiology
Diarrhea, Infantile - pathology
Down Syndrome
Down Syndrome - complications
Down Syndrome - pathology
Enterocytes - enzymology
Enterocytes - ultrastructure
Female
Hirschprung Disease
Hirschsprung Disease - complications
Hirschsprung Disease - pathology
Hirschsprung Disease - surgery
Humans
Infant
Intestinal Microvillous Atrophy
Intestinal Mucosa - enzymology
Intestinal Mucosa - ultrastructure
Jejunum - metabolism
Jejunum - pathology
Periodic Acid-Schiff Reaction
Treatment Outcome
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Summary:Intestinal microvillous disorders are an uncommon cause of severe diarrhea, with very poor prognosis. The authors report the case of a female infant with Down syndrome, aganglionic megacolon, severe diarrhea, and jejunal biopsy with ultrastructural changes consistent with microvillous atrophy. The patient condition improved after a colostomy performed in the setting of the treatment of Hirschprung disease.
ISSN:0191-3123
1521-0758
DOI:10.1080/01913120252934323