Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease
Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease
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DOI：
10.1016/s1569-1993(11)60006-4
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发表时间：
2011-06-01
影响因子：
5.2
通讯作者：
Colombo, Carla
中科院分区：
文献类型：
--
作者：
Debray, Dominique;Kelly, Deirdre;Colombo, Carla
大约 5-10% 的囊性纤维化 (CF) 患者在生命的第一个十年内会发展为多小叶性肝硬化。大多数 CF 患者随后出现门静脉高压症状并伴有并发症，主要是静脉曲张出血。肝衰竭通常发生在儿童年龄之后。建议每年进行肝病筛查，以检测症状前体征并开始熊去氧胆酸治疗，这可能会阻止疾病进展。如果存在以下至少两个变量，则应考虑肝病：体格检查异常、肝功能检查持续异常和病理超声检查。如果诊断有疑问，则需要进行肝活检。所有患有肝病的 CF 患者都需要每年进行随访，以评估肝硬化、门静脉高压或肝功能衰竭的发展情况。治疗重点应放在营养、预防出血和静脉曲张减压上。肺功能恶化是肝移植的一个重要考虑因素，特别是对于患有肝功能障碍或晚期门静脉高压的儿童。 (C) 2011 年欧洲囊性纤维化协会。由 Elsevier B.V. 出版。保留所有权利。
Approximately 5-10% of cystic fibrosis (CF) patients develop multilobular cirrhosis during the first decade of life. Most CF patients later develop signs of portal hypertension with complications, mainly variceal bleeding. Liver failure usually occurs later, after the paediatric age. Annual screening for liver disease is recommended to detect pre-symptomatic signs and initiate ursodeoxycholic acid therapy, which might halt disease progression. Liver disease should be considered if at least two of the following variables are present: abnormal physical examination, persistently abnormal liver function tests and pathological ultrasonography. If there is diagnostic doubt, a liver biopsy is indicated. All CF patients with liver disease need annual follow-up to evaluate the development of cirrhosis, portal hypertension or liver failure. Management should focus on nutrition, prevention of bleeding and variceal decompression. Deterioration of pulmonary function is an important consideration for liver transplantation, particularly in children with hepatic dysfunction or advanced portal hypertension. (C) 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.