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microvillous atrophy

Wednesday 23 November 2005

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

- Microvillous inclusion disease (microvillous atrophy). Ruemmele FM, Schmitz J, Goulet O. Orphanet J Rare Dis. 2006 Jun 26;1:22. PMID: 16800870

- [CD10 expression in a case of microvillous inclusion disease] Youssef N, M Ruemmele F, Goulet O, Patey N. Ann Pathol. 2004 Dec;24(6):624-7. French. PMID: 15785408

- Intractable diarrhea of infancy with congenital intestinal mucosa abnormalities: outcome of four cases. Gambarara M, Diamanti A, Ferretti F, Papadatou B, Knafelz D, Pietrobattista A, Castro M. Transplant Proc. 2003 Dec;35(8):3052-3. PMID: 14697977

- 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

- Dihydropyrimidinase deficiency and congenital microvillous atrophy: coincidence or genetic relation? Assmann B, Hoffmann GF, Wagner L, Bräutigam C, Seyberth HW, Duran M, Van Kuilenburg AB, Wevers R, Van Gennip AH. J Inherit Metab Dis. 1997 Sep;20(5):681-8. PMID: 9323563

- 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

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