Gallbladder and bile duct disease in Cystic Fibrosis.
Gallbladder and bile duct disease in Cystic Fibrosis.
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DOI：
10.1016/j.jcf.2017.07.006
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发表时间：
2017-11-01
期刊：
影响因子：
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通讯作者：
Debray, Dominique
中科院分区：
文献类型：
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作者：
Assis, David N;Debray, Dominique
囊性纤维化（CF）是由囊性纤维化跨膜传导受体（CFTR）突变引起的多器官、临床多样性疾病。意识到肺外表现，包括胃肠道和肝胆紊乱，是为CF患者提供高质量护理的一个越来越重要的部分。此外，胆道疾病，包括胆囊和胆管疾病，是CF的常见并发症。因此，对胆囊和胆道系统的全面了解和有效的临床评价是CF患者综合护理的一个重要方面，以防止未检测到的病理进展。这篇最佳实践文章总结了胆囊和胆管病理学的基础，描述了胆道疾病的背景和临床表现，并提供了为CF患者提供高质量护理的推荐方法。
Cystic fibrosis (CF) is a multi-organ, clinically diverse disorder caused by mutations in the cystic fibrosis transmembrane conductance receptor (CFTR). Awareness of extra-pulmonary manifestations, including gastrointestinal and hepatobiliary disturbances, is an increasingly important part of providing high-quality care to patients with CF. Furthermore, biliary disorders, including gallbladder and bile duct disease, are common complications of CF. Therefore, a thorough understanding and efficient clinical evaluation of the gallbladder and biliary tree is an important aspect of integrated care for the patient with CF in order to prevent progression of undetected pathology. This best practice article summarizes the basis for gallbladder and bile duct pathology, describes the context and clinical presentation of biliary disease, and provides recommended approaches to delivery of high-quality care for patients with CF.