Characteristics and outcome of primary sclerosing cholangitis associated with inflammatory bowel disease in Asian children
Characteristics and outcome of primary sclerosing cholangitis associated with inflammatory bowel disease in Asian children
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DOI：
10.1016/j.pedneo.2018.09.007
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发表时间：
2019-08-01
影响因子：
2.1
通讯作者：
Aw, Marion Margaret
中科院分区：
文献类型：
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作者：
Lee, Way Seah;Karthik, Sivaramakrishnan Venkatesh;Aw, Marion Margaret
工作背景：目前对亚洲儿童原发性硬化性胆管炎-炎症性肠病的临床特征和自然史的了解有限。我们描述了原发性硬化性胆管炎-炎症性肠病的表现特征和自然史，在一个东南亚儿童队列中观察到。方法：我们对新加坡和马来西亚的三个三级中心的儿童原发性硬化性胆管炎-炎症性肠病进行了回顾性分析。（男孩，58%;诊断时的中位年龄：6.3岁）（溃疡性结肠炎，n = 21;克罗恩病，n = 1;未分化，n = 2），63%（n = 15）在随访期间被诊断为结肠炎，21%（n = 5）表现为急性或慢性肝炎，17%（n = 4）同时出现。肝脏受累的疾病表型显示79%有硬化性胆管炎-自身免疫性肝炎重叠，54%有大胆管疾病，46%有小胆管疾病。所有患者均接受免疫抑制治疗。中位[+/- S.D.]随访4.7 [+/- 3.8]年，12.5%的患者肝酶正常，75%的患者持续存在疾病，12.5%的患者肝功能衰竭。肝硬化患者的比例从诊断时的13%增加到29%; 21%患有门脉高压症，17%患有肝功能障碍。1例患者需要肝移植。无移植存活率为95%。对于结肠炎，95%的患者在初次就诊时有全结肠炎，27%的患者有直肠保留，11%的患者有回肠炎。在最终审查时，67%的患者有静止期肠病伴免疫抑制。1例在3岁时诊断为UC伴全结肠炎的患者在22岁时发展为结直肠癌。所有患者均存活。结论：亚洲儿童原发性硬化性胆管炎-炎症性肠病的肝脏疾病严重程度不同。在免疫抑制的情况下，三分之二的患者有静止性肠病，但大多数患者有持续性胆管炎和进行性肝病。版权所有（C）2018，台湾儿科医学会.由爱思唯尔台湾有限公司出版。
Background: Current knowledge on the clinical features and natural history of childhood primary sclerosing cholangitis - inflammatory bowel disease in Asia is limited. We described the presenting features and natural history of primary sclerosing cholangitis-inflammatory bowel disease seen in a cohort of Southeast Asian children.Methods: We conducted a retrospective review of childhood primary sclerosing cholangitis-inflammatory bowel disease from three tertiary centers in Singapore and Malaysia.Results: Of 24 patients (boys, 58%; median age at diagnosis: 6.3 years) with primary sclerosing cholangitis-inflammatory bowel disease (ulcerative colitis, n = 21; Crohn's disease, n = 1; undifferentiated, n = 2), 63% (n = 15) were diagnosed during follow-up for colitis, and 21% (n = 5) presented with acute or chronic hepatitis, 17% (n = 4) presented simultaneously. Disease phenotype of liver involvement showed 79% had sclerosing cholangitis-autoimmune hepatitis overlap, 54% large duct disease, and 46% small duct disease. All patients received immunosuppression therapy. At final review after a median [+/- S.D.] duration follow-up of 4.7 [+/- 3.8] years, 12.5% patients had normal liver enzymes, 75% persistent disease, and 12.5% liver failure. The proportion of patients with liver cirrhosis increased from 13% at diagnosis to 29%; 21% had portal hypertension, and 17% had liver dysfunction. One patient required liver transplant. Transplant-free survival was 95%. For colitis, 95% had pancolitis, 27% rectal sparing, and 11% backwash ileitis at initial presentation. At final review, 67% patients had quiescent bowel disease with immunosuppression. One patient who had UC with pancolitis which was diagnosed at 3 years old developed colorectal cancer at 22 years of age. All patients survived.Conclusions: Liver disease in primary sclerosing cholangitis-inflammatory bowel disease in Asian children has variable severity. With immunosuppression, two-thirds of patients have quiescent bowel disease but the majority have persistent cholangitis and progressive liver disease. Copyright (C) 2018, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC.