Preferred Label : Microvillous inclusion disease;
ICD-11 definition : Microvillous inclusion disease (MVID) or microvillous atrophy is a congenital disorder
of the intestinal epithelial cells that presents with persistent life-threatening
watery diarrhea and is characterized 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.;
ICD-11 synonym : inclusion disease NOS; congenital microvillous atrophy;
Origin ID : 2137578537;
Automatic exact mappings (from CISMeF team)
Microvillous inclusion disease (MVID) or microvillous atrophy is a congenital disorder
of the intestinal epithelial cells that presents with persistent life-threatening
watery diarrhea and is characterized 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.