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囊性纤维化：肝胆疾病

Authors
Daniel H Leung, MD
Drucy Borowitz, MD
Section Editors
George B Mallory, MD
Elizabeth B Rand, MD
Deputy Editor
Alison G Hoppin, MD
Translators
史留斌, 主任医师，教授

引言

由于对囊性纤维化(cystic fibrosis, CF)的早期诊断、期望寿命延长，以及医护人员对CF的筛查和监测更加警惕，对囊性纤维化相关性肝病(cystic fibrosis-related liver disease, CFLD)的识别也正日益增多。严重CFLD患者常在儿童期发病，疾病往往进展迅速。较轻的CFLD可能难以诊断，因为患者可能保持无症状直到病程晚期，而且目前可行的筛查方法与疾病严重程度的相关性较差。早期发现CFLD，就能预测并治疗其并发症，包括营养不良、门静脉高压引起的静脉破裂出血和偶尔出现的肝衰竭。CF的其他肝胆并发症包括胆石症(胆石)、胆囊炎和微小胆囊。

本专题将讨论CFLD的临床表现、诊断和处理，相关的胆囊疾病也会进行简要讨论。与CF和肝胆疾病患者的处理相关的其他专题包括：

●(参见“Cystic fibrosis: Overview of gastrointestinal disease”)

●(参见“囊性纤维化相关糖尿病”)

●(参见“Cystic fibrosis: Clinical manifestations and diagnosis”)

                        

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Literature review current through: 2017-06 . | This topic last updated: 2015-10-12.
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