Classen M, Götze H, Richter H J, Bender S
Childrens Hospital, Frankfurt/Main, F.R.G.
J Pediatr Gastroenterol Nutr. 1987 Mar-Apr;6(2):197-202. doi: 10.1097/00005176-198703000-00005.
Primary sclerosing cholangitis (PSC) in eight children, five males and three females between the ages of 4 and 13 years, presented with minimal clinical symptoms and few signs of liver disease. Diagnosis was made by a highly characteristic histology in all and additional endoscopic retrograde cholangiography (ERC) in four patients. Six children suffered from concomitant inflammatory bowel disease (IBD). Laboratory abnormalities consisted of mild elevation of transaminases and alkaline phosphatase, with marked elevation of immunoglobulin G in seven and detection of anti-nuclear antibodies in four of the eight children. In all cases, needle liver biopsy specimens revealed portal tracts considerably expanded by edema and chronic inflammation diagnostic of nonsuppurative cholangitis and nonsuppurative fibrosing pericholangitis. ERC showed decreased arborization in the whole biliary tree. In two patients, changes were confined to the intrahepatic portion of the biliary tract. No specific drug therapy was given to our patients. Those with concomitant IBD were treated with salazosulfapyridine (SASP). The clinical course has been mild in all patients up to now. It is concluded that PSC may be much more frequent in childhood than was considered before, especially in children with IBD.
8名儿童（年龄在4至13岁之间，5名男性，3名女性）患有原发性硬化性胆管炎（PSC），临床症状轻微，肝病体征较少。所有患儿均通过高度特征性的组织学检查确诊，4例患儿还进行了额外的内镜逆行胆管造影（ERC）检查。6名儿童同时患有炎症性肠病（IBD）。实验室检查异常包括转氨酶和碱性磷酸酶轻度升高，8名儿童中有7名免疫球蛋白G显著升高，4名检测到抗核抗体。在所有病例中，肝脏穿刺活检标本显示门管区因水肿和慢性炎症而明显增宽，诊断为非化脓性胆管炎和非化脓性纤维性胆管周炎。ERC显示整个胆管树分支减少。2例患者的改变局限于肝内胆管部分。我们的患者未接受特殊药物治疗。合并IBD的患者接受柳氮磺胺吡啶（SASP）治疗。截至目前，所有患者的临床病程均较轻微。得出结论，PSC在儿童期可能比以前认为的更为常见，尤其是在患有IBD的儿童中。
