Microvillous inclusion disease (Q107203)

Microvillous inclusion disease (MVID) or microvillous atrophy is a congenital disorder of the intestinal epithelial cells that presents with persistent life-threatening watery diarrhoea and is characterised by morphological enterocyte abnormalities. This is a rare genetic disorder that is inherited in an autosomal recessive pattern. It is caused by a congenital lack of apical microvilli in the epithelial cells of the small intestine, however, it usually lacks the intraepithelial lymphocytic infiltration characteristic of celiac sprue and stains positive for carcinoembryonic antigen (CEA). MVID manifests either in the first days of life (early-onset form) or in the first two months (late-onset form) of life.