Small bowel (small intestine) - Microvillus inclusion disease

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Small bowel (small intestine)

Malabsorption

Microvillus inclusion disease

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 14 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Also called congenital or familial microvillous atrophy
● Disorder of intestinal brush border that causes intractable watery diarrhea with steatorrhea in infants
● Patients require total parental nutrition, and rarely live beyond age 2 years
● Villous atrophy may be due to apoptotic cell loss (Hum Pathol 2000;31:1404)

Treatment
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● Small bowel transplant

Micro description
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● Severe villous abnormality with crypt hypoplasia, resembling celiac sprue but without lymphocytosis
● Increased enterocyte apoptosis and proliferation, bubbly vacuolated apical cytoplasm with extensive or patchy absence of brush border, absence of inflammation (Ultrastruct Pathol 2010;34:327)

Micro images
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Various images

Positive stains
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● CD10 (Am J Surg Pathol 2002;26:902), PAS, polyclonal CEA, alkaline phosphatase (cytoplasmic staining vs. linear brush border staining in normals)
● Vacuoles - PAS, CEA
● Cytoplasmic CD10 staining of absorptive colonocytes can aid in the diagnosis in situations where only colonic biopsy could be obtained (Am J Surg Pathol 2010;34:970)

Electron microscopy description
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● Abnormal microvillus structures at luminal border of enterocytes
● Apical intracytoplasmic inclusions lined by microvilli

Electron microscopy images
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Neonatal congenital microvillus atrophy

End of Small bowel (small intestine) > Malabsorption > Microvillus inclusion disease

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