Schofield D E, Agostini R M, Yunis E J
Department of Pathology, Children's Hospital of Pittsburgh, Pennsylvania.
Am J Clin Pathol. 1992 Jul;98(1):119-24. doi: 10.1093/ajcp/98.1.119.
A 3-year-old girl of Navajo heritage had intractable diarrhea beginning at 4 days of age and resulting in long-term hyperalimentation. Investigation before multivisceral transplantation included biopsies of the rectum, stomach, duodenum, and liver. The diagnosis of microvillus inclusion disease was established by documentation of microvillus inclusions in duodenal epithelial cells. A trial of somatostatin therapy was ineffective in controlling the diarrhea. Subsequently, a multivisceral organ transplant provided a unique opportunity to establish the gastrointestinal extent of involvement of this disease. Ultrastructural microvillus inclusions were identified in the duodenum, jejunum, ileum, and colon, but not in the gallbladder. A few inclusions also were documented in gastric antral epithelial cells. Alkaline phosphatase stains performed on paraffin-embedded material showed a few inclusions in the antrum of the stomach and many inclusions throughout the small intestine, primarily in surface epithelial cells but also in upper crypt cells.
一名具有纳瓦霍族血统的3岁女孩自4日龄起出现顽固性腹泻，导致长期接受胃肠外营养。多脏器移植前的检查包括直肠、胃、十二指肠和肝脏活检。十二指肠上皮细胞中存在微绒毛包涵体，据此确诊为微绒毛包涵体病。生长抑素治疗试验未能有效控制腹泻。随后，多脏器移植为确定该疾病在胃肠道的累及范围提供了独特机会。在十二指肠、空肠、回肠和结肠中发现了超微结构微绒毛包涵体，但胆囊中未发现。胃窦上皮细胞中也发现了一些包涵体。对石蜡包埋材料进行的碱性磷酸酶染色显示，胃窦有一些包涵体，整个小肠有许多包涵体，主要存在于表面上皮细胞，但也存在于隐窝上部细胞中。
