Chong S K, Wright V M, Nishigame T, Raafat F, Isaacs D, Morson B C, Walker-Smith J A
King's College Hospital, London, England.
J Pediatr Gastroenterol Nutr. 1988 Jul-Aug;7(4):622-7.
A brother and sister of first cousin Pakistani parents presented with recurrent mouth ulcers and chronic diarrhoea in the neonatal period. Diarrhoea persisted in spite of treatment with oral prednisolone and sulphasalazine. Both children required subtotal colectomy with ileostomy. Histopathology of the resected colons was virtually identical and showed multiple deep "flask"-shaped ulcers, often penetrating to the serosa, in the presence of chronic inflammation, but without any of the characteristic histological features of Crohn's disease. The appearance closely resembled the colitis of Beh,cet's syndrome. Both children (aged 6 and 3.5 years, respectively) have continued to have recurrent perianal disease with intermittent bloody diarrhoea since the operation. Apart from the initial symptoms of oral aphthous ulcerations in both children, no other major criteria have developed so far. Intestinal Behçet's syndrome should be considered in the differential diagnosis of chronic inflammatory bowel disease in childhood.
一对表亲是巴基斯坦人的兄妹在新生儿期出现反复口腔溃疡和慢性腹泻。尽管使用口服泼尼松龙和柳氮磺胺吡啶治疗,腹泻仍持续存在。两个孩子都需要进行次全结肠切除术并造口。切除结肠的组织病理学几乎相同,显示有多个深部“烧瓶”状溃疡,常穿透至浆膜层,伴有慢性炎症,但没有克罗恩病的任何典型组织学特征。其表现与白塞氏综合征的结肠炎极为相似。自手术以来,两个孩子(分别为6岁和3.5岁)持续出现反复肛周疾病并伴有间歇性血性腹泻。除了两个孩子最初的口腔阿弗他溃疡症状外,目前尚未出现其他主要标准。在儿童慢性炎症性肠病的鉴别诊断中应考虑肠道白塞氏综合征。