Iancu Theodore C, Mahajnah Muhammad, Manov Irena, Shaoul Ron
Pediatric Research and Electron Microscopy Unit, The Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Ultrastruct Pathol. 2007 May-Jun;31(3):173-88. doi: 10.1080/01913120701350712.
Microvillous inclusion disease (MVID) is a congenital, usually neonatal, autosomal recessive condition manifested by severe, prolonged secretory diarrhea. Intestinal biopsies reveal extensive microvilli abnormalities, typical inclusions and vesicles mainly of the apical-luminal enterocytes and colonocytes. Although diagnosis can be suspected by special stains of the mucosa (PAS, CD10), the definitive diagnosis, recommended in view of potential intestinal transplantation, requires electron microscopy. In view of the marked variability of ultrastructural changes, extensive illustration is considered valuable for diagnosis. While the pathogenesis is still unknown, a number of images illustrate the suspected "arrested-trafficking" hypothesis of microvillous abnormalities. Others micrographs support the "engulfing" mechanism of inclusion formation. The electron micrographs should help ultrastructural diagnosis in this heterogeneous disease and can confirm diagnosis even in the absence of the typical inclusions.
微绒毛包涵体病（MVID）是一种先天性疾病，通常在新生儿期发病，为常染色体隐性遗传，表现为严重的持续性分泌性腹泻。肠道活检显示广泛的微绒毛异常，典型的包涵体和囊泡主要见于顶端腔面的肠上皮细胞和结肠细胞。尽管通过黏膜特殊染色（PAS、CD10）可怀疑诊断，但鉴于可能需要进行肠道移植，明确诊断需要电子显微镜检查。鉴于超微结构变化的显著变异性，大量图示对于诊断很有价值。虽然发病机制尚不清楚，但一些图像说明了微绒毛异常的疑似“转运停滞”假说。其他显微照片支持包涵体形成的“吞噬”机制。电子显微镜照片应有助于对这种异质性疾病进行超微结构诊断，即使在没有典型包涵体的情况下也能确诊。
