Paediatric-onset autoimmune liver disease: Insights from a monocentric experience.

IF 4 3区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY Digestive and Liver Disease Pub Date : 2024-10-15 DOI:10.1016/j.dld.2024.09.020
Franco Curci, Chiara Rubino, Mariangela Stinco, Simona Carrera, Sandra Trapani, Elisa Bartolini, Giuseppe Indolfi
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Abstract

Background: Autoimmune liver disease (AILD) encompasses autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC) and primary sclerosing cholangitis (PSC). A unified disease process evolving over time through these entities has been recently suggested. From this perspective, this study aimed to compare the characteristics of childhood-onset AILD at baseline and after a medium-to-long term follow-up period.

Methods: Paediatric-onset cases of AILD diagnosed between 1992 and 2023 at a tertiary-care centre were reviewed. Patients transitioned to adult-care by the time of data collection were asked for clinical updates.

Results: Fifty-five patients were included (AIH = 20, ASC =22, PSC =13). AIH, ASC and PSC exhibited increasing age at the onset (AIH to PSC, p < 0.01). The area under the receiver operating characteristic curve for gamma-glutamyltranspeptidase (GGT) combined with alkaline phosphatase/aspartate aminotransferase (ALP/AST) ratio in predicting sclerosing cholangitis was 0.94, with a sensitivity of 86 % and a specificity of 94 %. At the last follow-up (median duration 5,8 years, interquartile range [IQR] 2,9-10,2, n = 45), 15 patients (33 %) developed portal hypertension, 2 patients (4 %) underwent liver transplantation, no patient died.

Conclusion: A cohort of childhood-onset AILD managed at a single centre reveals a temporal trend in the onset of AIH, ASC and PSC, with progressively older ages. Elevated GGT levels combined with a high ALP/AST ratio predict the diagnosis of sclerosing cholangitis. The occurrence of liver-related adverse events in one-third of patients highlights the progressive nature of paediatric-onset AILD.

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儿科自身免疫性肝病:单中心经验的启示
背景:自身免疫性肝病(AILD)包括自身免疫性肝炎(AIH)、自身免疫性硬化性胆管炎(ASC)和原发性硬化性胆管炎(PSC)。最近有人提出,通过这些实体随时间演变的疾病过程是统一的。从这个角度出发,本研究旨在比较儿童期发病的 AILD 在基线和中长期随访后的特征:方法:研究人员回顾了一家三级医疗中心在 1992 年至 2023 年期间确诊的儿童型 AILD 病例。在收集数据时,已转入成人护理的患者被要求提供最新临床资料:结果:共纳入55名患者(AIH=20,ASC=22,PSC=13)。AIH、ASC和PSC患者的发病年龄呈上升趋势(从AIH到PSC,P < 0.01)。γ-谷氨酰转肽酶(GGT)与碱性磷酸酶/天门冬氨酸氨基转移酶(ALP/AST)比值预测硬化性胆管炎的接收者操作特征曲线下面积为0.94,敏感性为86%,特异性为94%。在最后一次随访中(中位数病程 5.8 年,四分位数间距 [IQR] 2.9-10.2 年,n = 45),15 名患者(33%)出现门脉高压,2 名患者(4%)接受了肝移植,没有患者死亡:结论:在一个中心接受治疗的一组儿童期发病的 AILD 患者显示,AIH、ASC 和 PSC 的发病年龄有逐渐增大的趋势。GGT水平升高加上ALP/AST比值升高可预测硬化性胆管炎的诊断。三分之一的患者会出现与肝脏相关的不良反应,这凸显了儿科发病型 AILD 的渐进性。
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来源期刊
Digestive and Liver Disease
Digestive and Liver Disease 医学-胃肠肝病学
CiteScore
6.10
自引率
2.20%
发文量
632
审稿时长
19 days
期刊介绍: Digestive and Liver Disease is an international journal of Gastroenterology and Hepatology. It is the official journal of Italian Association for the Study of the Liver (AISF); Italian Association for the Study of the Pancreas (AISP); Italian Association for Digestive Endoscopy (SIED); Italian Association for Hospital Gastroenterologists and Digestive Endoscopists (AIGO); Italian Society of Gastroenterology (SIGE); Italian Society of Pediatric Gastroenterology and Hepatology (SIGENP) and Italian Group for the Study of Inflammatory Bowel Disease (IG-IBD). Digestive and Liver Disease publishes papers on basic and clinical research in the field of gastroenterology and hepatology. Contributions consist of: Original Papers Correspondence to the Editor Editorials, Reviews and Special Articles Progress Reports Image of the Month Congress Proceedings Symposia and Mini-symposia.
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