Lachaux A, Loras-Duclaux I, Bouvier R
Department of Paediatric Gastroenterology, Hôspital Edouard Herriot, Lyon, France.
Virchows Arch. 1998 Nov;433(5):481-5. doi: 10.1007/s004280050277.
In two brothers with autoimmune enteropathy there was total villous atrophy in the small intestine and marked lymphoid cell infiltration in the lamina propria of the entire digestive tract, discovered at autopsy in one of these patients. In addition, the pancreas showed diffuse interstitial infiltration by lymphocytes. The liver was enlarged, with extensive haematopoiesis and cholestasis. Similar lesions in the digestive tract were noticed in the second boy, but on immunosuppressive therapy his diarrhoea gradually disappeared. When he was 16 months of age, percutaneous biopsies showed moderately aggressive chronic hepatitis and a focal interstitial lymphoid infiltrate in the kidney. After 3 years of immunosuppressive therapy (prednisone, cyclosporin), the child ate well and total parenteral nutrition was discontinued. The intestinal lesions had regressed but fibrotic lesions of the liver persisted.
在两名患有自身免疫性肠病的兄弟中，其中一名患者尸检发现小肠存在完全性绒毛萎缩，整个消化道固有层有明显的淋巴细胞浸润。此外，胰腺显示淋巴细胞弥漫性间质浸润。肝脏肿大，有广泛的造血和胆汁淤积。第二名男孩的消化道也发现了类似病变，但在免疫抑制治疗后，他的腹泻逐渐消失。在他16个月大时，经皮活检显示为中度侵袭性慢性肝炎，肾脏有局灶性间质淋巴细胞浸润。经过3年的免疫抑制治疗（泼尼松、环孢素），孩子食欲良好，停止了全胃肠外营养。肠道病变已消退，但肝脏的纤维化病变仍然存在。
