Background and Aims Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection and monitoring of hepatobiliary complications in CF were published in 1999. The CF Foundation assembled a committee to evaluate research advances and formulate revised guidelines for CF-associated liver disease. Approach A committee of hepatologists, gastroenterologists, pulmonologists, pharmacist, nurse, dietitian, individual with CF, and parent of a child with CF devised "population, intervention, comparison, and outcome" (PICO) questions regarding hepatobiliary disease in CF. PubMed literature searches were performed for each PICO question. Recommendations were voted on with 80% agreement required to approve a recommendation. Public comment on initial recommendations was solicited prior to formulation of final recommendations. Results 31 PICO questions were assembled, 6,401 manuscripts were title screened for relevance, with 1,053 manuscripts undergoing detailed full text review. Seven recommendations were approved for screening, 13 for monitoring of existing disease, and 14 for treatment of CF-associated hepatobiliary involvement or advanced liver disease. One recommendation on liver biopsy did not meet the 80% threshold. One recommendation on screening ultrasound was revised and re-voted on. Conclusions Through a multidisciplinary committee and public engagement, we have assembled updated recommendations and guidance on screening, monitoring and treatment of CF-associated hepatobiliary involvement and advanced liver disease. While research gaps remain, we anticipate that these recommendations will lead to improvements in CF outcomes through earlier detection and increased evidence-based approaches to monitoring and treatment.

中文翻译：

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囊性纤维化筛查、评估和肝胆疾病管理共识建议。

背景和目的囊性纤维化（CF）可能引起一系列肝胆并发症，包括门静脉高压、多小叶性肝硬化和肝衰竭。目前关于 CF 肝胆并发症检测和监测的指南于 1999 年发布。CF 基金会组建了一个委员会来评估研究进展并制定 CF 相关肝病的修订指南。方法 由肝病学家、胃肠病学家、肺病学家、药剂师、护士、营养师、CF 患者和 CF 儿童家长组成的委员会设计了有关 CF 肝胆疾病的“人群、干预、比较和结果”(PICO) 问题。对每个 PICO 问题都进行了 PubMed 文献检索。对建议进行了投票，需要 80% 的同意才能批准建议。在制定最终建议之前，已征求公众对初步建议的意见。结果 汇集了 31 个 PICO 问题，对 6,401 份手稿的相关性进行了标题筛选，其中 1,053 份手稿进行了详细的全文审查。批准了 7 项筛查建议，13 项用于监测现有疾病，14 项用于治疗 CF 相关肝胆受累或晚期肝病。一项关于肝活检的建议未达到 80% 的阈值。一项关于超声筛查的建议经过修改并重新投票。结论 通过多学科委员会和公众参与，我们收集了关于 CF 相关肝胆受累和晚期肝病筛查、监测和治疗的最新建议和指南。尽管研究差距仍然存在，但我们预计这些建议将通过早期检测和增加基于证据的监测和治疗方法来改善 CF 的结果。