衔接儿科肝病成人护理:胃肠病学家需要知道什么?

D. Kelly
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引用次数: 1

摘要

医学和外科治疗的进步意味着大量患有以前致命肝病的儿童能够存活到成年。特别是,80%的移植受者现在存活超过20年。治疗成人患者的胃肠病学家和肝病学家需要了解起源于婴儿期的疾病的临床管理和并发症,如胆道闭锁、进行性家族性肝内胆汁淤积症、Alagille综合征和代谢性疾病,如遗传性酪氨酸血症1型。他们需要熟悉儿童肝移植的长期后果,如肾功能衰竭、复发性疾病、骨质疏松症和移植后恶性肿瘤,特别是移植后淋巴增生性疾病,其表现和发展与成人移植受者不同。儿童期疾病的幸存者需要采取与18岁以后出现疾病的年轻人不同的方法。成人医生需要考虑这些年轻人的情感、社会和性健康,并意识到在门诊预约和用药方面的高不依从率,以及移植物损失的影响,特别是在过渡到成人服务之后。为这些年轻人制定适当的过渡性护理是基于儿科和成人界面的有效合作,这是21世纪儿科和成人提供者面临的一项重大挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Bridging Paediatric Liver Diseases to Adult Care: What Does the Gastroenterologist Need to Know?
Advances in medical and surgical therapy mean that significant numbers of children with previously fatal liver disease are surviving into adult life. In particular, 80% of transplant recipients now survive for over 20 years. Gastroenterologists and hepatologists who treat adult patients need to be aware of the clinical management and complications of diseases originating in infancy, such as biliary atresia, progressive familial intrahepatic cholestasis, Alagille syndrome, and metabolic diseases such as hereditary tyrosinemia type 1. They need to be familiar with the long-term consequences of liver transplantation in childhood, e.g. renal failure, recurrent disease, osteoporosis, and post-transplant malignancies, especially post-transplant lymphoproliferative disease, which differs in presentation and evolution from adult transplant recipients. Survivors of childhood illness require a different approach to that for young adults presenting after 18 years of age. Adult physicians need to consider the emotional, social, and sexual health of these young people, and be aware of the high rate of non-adherence, both for clinic appointments and medication, as well as the implications for graft loss, particularly after transition to adult services. Developing adequate transitional care for these young people is based on effective collaboration at the paediatric–adult interface and is a major challenge for paediatric and adult providers alike in the 21st century.
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