Pathophysiology in Microvillus inclusion disease
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Pathophysiology in Microvillus inclusion disease. / Reinshagen, K; Naim, H; Heusipp, G; Zimmer, K-P.
in: Z GASTROENTEROL, Jahrgang 44, Nr. 8, 08.2006, S. 667-71.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - Pathophysiology in Microvillus inclusion disease
AU - Reinshagen, K
AU - Naim, H
AU - Heusipp, G
AU - Zimmer, K-P
PY - 2006/8
Y1 - 2006/8
N2 - Microvillus inclusion disease (MID) is a congenital disorder with the clinical signs of watery diarrhea often beginning in the first days of life. The main pathological features of the disease include a villus atrophy and an accumulation of periodic acid-Schiff (PAS)-positive material within the apical cytoplasm of enterocytes on the light microscopy level. Electron microscopic criteria are pathognomonic consisting of an increased amount of secretory granules preferentially in crypt epithelial cells and of the presence of microvillus inclusion bodies (MIBs) which are most frequently found in villus enterocytes. Until now the basic molecular defects have not been disclosed completely. In this review we discuss the actual pathogenetic hypothesis and the therapeutic options besides small bowel transplantation.
AB - Microvillus inclusion disease (MID) is a congenital disorder with the clinical signs of watery diarrhea often beginning in the first days of life. The main pathological features of the disease include a villus atrophy and an accumulation of periodic acid-Schiff (PAS)-positive material within the apical cytoplasm of enterocytes on the light microscopy level. Electron microscopic criteria are pathognomonic consisting of an increased amount of secretory granules preferentially in crypt epithelial cells and of the presence of microvillus inclusion bodies (MIBs) which are most frequently found in villus enterocytes. Until now the basic molecular defects have not been disclosed completely. In this review we discuss the actual pathogenetic hypothesis and the therapeutic options besides small bowel transplantation.
KW - Diarrhea/congenital
KW - Humans
KW - Inclusion Bodies/pathology
KW - Malabsorption Syndromes/pathology
KW - Microvilli/pathology
U2 - 10.1055/s-2006-926903
DO - 10.1055/s-2006-926903
M3 - SCORING: Review article
C2 - 16902898
VL - 44
SP - 667
EP - 671
JO - Z GASTROENTEROL
JF - Z GASTROENTEROL
SN - 0044-2771
IS - 8
ER -