Childhood Cholestatic Liver Diseases that Persist Into Adulthood

胆道闭锁 医学 进行性家族性肝内胆汁淤积症 胆汁淤积 阿拉吉尔综合征 肝移植 新生儿胆汁淤积症 肝病 胆汁酸 疾病 原发性硬化性胆管炎 儿科 内科学 移植 胃肠病学
作者
Alvin P. Chan,Robert S. Venick
出处
期刊:Journal of Clinical Gastroenterology [Lippincott Williams & Wilkins]
卷期号:57 (7): 686-693
标识
DOI:10.1097/mcg.0000000000001850
摘要

Children with cholestatic liver diseases are increasingly living into adulthood, thanks to innovations in medical and surgical therapies. The excellent outcomes observed in pediatric liver transplantation for diseases, such as biliary atresia, have transformed the life trajectory of children born with once-fatal liver diseases. The evolution of molecular genetic testing, has helped expedite the diagnosis of other cholestatic disorders, improving the clinical management, disease prognosis, and family planning for inherited disorders, such as progressive familial intrahepatic cholestasis and bile acid synthesis disorders. The expanding list of therapeutics, including bile acids and the newer ileal bile acid transport inhibitors, has also helped slow the progression of disease and improve the quality of life for certain diseases, like Alagille syndrome. More and more children with cholestatic disorders are expected to require care from adult providers familiar with the natural history and potential complications of these childhood diseases. The aim of this review is to bridge the gap between pediatric and adult care in children with cholestatic disorders. The present review addresses the epidemiology, clinical features, diagnostic testing, treatment, prognosis, and transplant outcomes of 4 hallmark childhood cholestatic liver diseases: biliary atresia, Alagille syndrome, progressive familial intrahepatic cholestasis, and bile acid synthesis disorders.
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