Microvillous inclusion disease: Ultrastructural variability
Microvillous inclusion disease: Ultrastructural variability
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DOI：
10.1080/01913120701350712
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发表时间：
2007-01-01
影响因子：
1
通讯作者：
Shaoul, Ron
中科院分区：
文献类型：
--
作者：
Lancu, Theodore C.;Mahajnah, Muhammad;Shaoul, Ron
微绒毛包涵体病（MVID）是一种先天性，通常是新生儿，常染色体隐性遗传疾病，表现为严重的，长期的分泌性腹泻。肠活检显示广泛的微绒毛异常，典型的包涵体和囊泡主要是顶端腔肠上皮细胞和结肠细胞。虽然可以通过粘膜的特殊染色（PAS，CD 10）来怀疑诊断，但鉴于潜在的肠移植，推荐的明确诊断需要电子显微镜检查。鉴于超微结构变化的显著可变性，广泛的图示被认为对诊断有价值。虽然发病机制仍然是未知的，一些图像说明了可疑的“交通受阻”假说的微绒毛异常。其他显微照片支持“吞没”机制的夹杂物形成。电子显微镜有助于这种异质性疾病的超微结构诊断，即使在缺乏典型内含物的情况下也能确诊。
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.