Synonyms
Congenital microvillous atrophy; Davidson disease; Familial microvillous atrophy; Intestinal microvillous atrophy; Intestinal microvillous dystrophy; Microvillous atrophy
Definition
Microvillous inclusion disease is an uncommon congenital enteropathy characterized by severe, intractable diarrhea within the first weeks of life. The affected infants have a clinical presentation including the presence of diarrhea for more than 2 weeks, severe nutritional malabsorption, and negative stool cultures. The disease was first described by Davidson in 1978 severe secretory diarrhea occurring during the first week of life with villous atrophy in the intestinal biopsy.
Clinical Features
Incidence
Rare condition. More than 50 cases of MID have been reported so far in the English literature.
Age
It is a disease of neonates and infants in the first 6 months of life. MID manifests either in the first days (early-onset form) or in the first 2 months (late-onset form) of life. The peak age of...
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References and Further Reading
Cutz, E., Sherman, P. M., & Davidson, G. P. (1997). Enteropathies associated with protracted diarrhea of infancy: Clinicopathological features, cellular and molecular mechanisms. Pediatric Pathology & Laboratory Medicine, 17(3), 335–368.
Groisman, G. M., Amar, M., & Livne, E. (2002). CD10, a valuable tool for the light microscopic diagnosis of microvillous inclusion disease (familial microvillous atrophy). The American Journal of Surgical Pathology, 26(7), 902–907.
Khubchandani, S. R., Vohra, P., Chitale, A. R., & Sidana, P. (2011). Microvillous inclusion disease-an ultrastructural diagnosis: With a review of the literature. Ultrastructural Pathology, 35, 87–91.
Shaila, R. K., Pankaj, V., Arun, R. C., & Poonam, S. (2011). Microvillous inclusion disease-an ultrastructural diagnosis: With a review of the literature. Ultrastructural Pathology, 35, 87–91.
Szperl, A. M., Golachowska, M. R., Bruinenberg, M., Prekeris, R., Thunnissen, A. M., Karrenbeld, A., Dijkstra, G., Hoekstra, D., Mercer, D., Ksiazyk, J., Wijmenga, C., Wapenaar, M. C., Rings, E. H., & van IJzendoorn, S. C. (2011). Functional characterization of mutations in the myosin Vb gene associated with microvillus inclusion disease. Journal of Pediatric Gastroenterology and Nutrition, 52, 307–313.
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Ensari, A. (2017). Microvillous Inclusion Disease. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1511
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1511
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