Microvillous inclusion disease: ultrastructural variability

TC Iancu, M Mahajnah, I Manov… - Ultrastructural …, 2007 - Taylor & Francis
TC Iancu, M Mahajnah, I Manov, R Shaoul
Ultrastructural Pathology, 2007Taylor & Francis
Microvillous inclusion disease (MVID) is a congenital, usually neonatal, autosomal recessive
condition manifested by severe, prolonged secretory diarrhea. Intestinal biopsies reveal
extensive microvilli abnormalities, typical inclusions and vesicles mainly of the apical-
luminal enterocytes and colonocytes. Although diagnosis can be suspected by special
stains of the mucosa (PAS, CD10), the definitive diagnosis, recommended in view of
potential intestinal transplantation, requires electron microscopy. In view of the marked …
Microvillous inclusion disease (MVID) is a congenital, usually neonatal, autosomal recessive condition manifested by severe, prolonged secretory diarrhea. Intestinal biopsies reveal extensive microvilli abnormalities, typical inclusions and vesicles mainly of the apical-luminal enterocytes and colonocytes. Although diagnosis can be suspected by special stains of the mucosa (PAS, CD10), the definitive diagnosis, recommended in view of potential intestinal transplantation, requires electron microscopy. In view of the marked variability of ultrastructural changes, extensive illustration is considered valuable for diagnosis. While the pathogenesis is still unknown, a number of images illustrate the suspected “arrested-trafficking” hypothesis of microvillous abnormalities. Others micrographs support the “engulfing” mechanism of inclusion formation. The electron micrographs should help ultrastructural diagnosis in this heterogeneous disease and can confirm diagnosis even in the absence of the typical inclusions.
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