microvillous atrophy
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Microvillous inclusion disease is a rare congenital defect of intestinal brush border of unknown aetiology. An autosomal recessive inheritance is suggested by cases occurring in siblings and high incidence of consanguinity. The prognosis of the disease is extremely poor, as life can be sustained only by total parenteral nutrition. Combined bowel-liver or bowel transplantation is regarded as the only potentially life-saving therapy.
Synopsis
PAS appearance
Immunochemistry
CD10 expression (15785408)
See also
neonatal enteropathies
intractable diarrhea of infancy
References
Groisman GM, Amar M, Livne E. CD10: a valuable tool for the light microscopic diagnosis of microvillous inclusion disease (familial microvillous atrophy). Am J Surg Pathol. 2002 Jul;26(7):902-7. PMID: 12131157
Mierau GW, Wills EJ, Wyatt-Ashmead J, Hoffenberg EJ, Cutz E. Microvillous inclusion disease: report of a case with atypical features. Ultrastruct Pathol. 2001 Nov-Dec;25(6):517-21. PMID: 11783915
Groisman GM, Sabo E, Meir A, Polak-Charcon S. Enterocyte apoptosis and proliferation are increased in microvillous inclusion disease (familial microvillous atrophy). Hum Pathol. 2000 Nov;31(11):1404-10. PMID: 11112216
Patey N, Scoazec JY, Cuenod-Jabri B, Canioni D, Kedinger M, Goulet O, Brousse N. Distribution of cell adhesion molecules in infants with intestinal epithelial dysplasia (tufting enteropathy). Gastroenterology. 1997 Sep;113(3):833-43. PMID: 9287975
Raafat F, Green NJ, Nathavitharana KA, Booth IW. Intestinal microvillous dystrophy: a variant of microvillous inclusion disease or a new entity? Hum Pathol. 1994 Nov;25(11):1243-8. PMID: 7959671
Nathavitharana KA, Green NJ, Raafat F, Booth IW. Siblings with microvillous inclusion disease. Arch Dis Child. 1994 Jul;71(1):71-3. PMID: 8067796
Groisman GM, Ben-Izhak O, Schwersenz A, Berant M, Fyfe B. The value of polyclonal carcinoembryonic antigen immunostaining in the diagnosis of microvillous inclusion disease. Hum Pathol. 1993 Nov;24(11):1232-7. PMID: 8244323
Jaffe R. Microvillous inclusion disease. Am J Surg Pathol. 1992 Nov;16(11):1129. PMID: 1335213
Bell SW, Kerner JA Jr, Sibley RK. Microvillous inclusion disease. The importance of electron microscopy for diagnosis. Am J Surg Pathol. 1991 Dec;15(12):1157-64. PMID: 1660676