Villin Immunohistochemistry Is a Reliable Method for Diagnosing Microvillus Inclusion Disease
Autor: | Kyle C. Kurek, Athos Bousvaros, Harry P.W. Kozakewich, Jeff Goldsmith, Nick Shillingford, Lisa A. Teot, Antonio R. Perez-Atayde, Monica L. Calicchio, Theonia K. Boyd |
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Rok vydání: | 2015 |
Předmět: |
Male
Pathology medicine.medical_specialty Brush border Cytoplasmic inclusion macromolecular substances Periodic acid–Schiff stain digestive system Stain Pathology and Forensic Medicine Malabsorption Syndromes Mucolipidoses Humans Medicine Child Retrospective Studies Microvilli biology business.industry Microfilament Proteins Infant Immunohistochemistry Microvillus Staining medicine.anatomical_structure Child Preschool biology.protein Female Surgery Anatomy Villin business Biomarkers |
Zdroj: | American Journal of Surgical Pathology. 39:245-250 |
ISSN: | 0147-5185 |
Popis: | Microvillus inclusion disease (MVID) is a rare congenital disorder that manifests early in infancy as intractable watery diarrhea. The entity is characterized morphologically by a deficient brush border and apical cytoplasmic inclusions within absorptive cells (enterocytes) due to misplaced assembly of brush border proteins. The diagnosis is based upon histopathology, special stains, immunohistochemistry (IHC), and ultimately upon electron microscopy. Currently, the periodic acid-Schiff stain (PAS) and CD10 IHC are commonly used as adjuncts, but in addition to brush border structures, they stain a variety of apical cytoplasmic inclusions and organelles, thereby interfering with recognition of microvillus inclusions. Villin is a protein that specifically binds to the actin core bundle of microvilli. We utilized villin IHC in formalin-fixed paraffin-embedded gastrointestinal biopsies from 6 patients with MVID, 5 with celiac disease, and 17 children with normal intestinal biopsies and compared the results with those obtained with CD10 IHC and PAS staining. All MVID cases had confirmatory electron microscopy at the time of diagnosis. Villin immunoreactivity was restricted to the brush border in the control groups. In MVID, villin IHC showed attenuation or loss of the surface brush border and also highlighted the cytoplasmic microvillus inclusions with clarity. In MVID, CD10 IHC and the PAS stain also showed attenuation or loss of the surface brush border, but staining of a variety of cytoplasmic structures largely obscured the microvillus inclusions. In sum, villin IHC is a reliable and superior adjunct in the diagnosis of MVID. Study of additional cases will determine whether villin IHC would obviate the need for electron microscopic confirmation. |
Databáze: | OpenAIRE |
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