BACKGROUND Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. METHODS The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. RESULTS The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues. CONCLUSIONS These recommendations are intended to be paired with previously published management guidelines for the overall CF population, with the objective of reducing practice variability and improving overall care, quality of life, and survival in those with ACFLD.

中文翻译：

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囊性纤维化基金会关于晚期囊性纤维化肺病患者护理的共识指南

背景晚期囊性纤维化肺病（ACFLD）很常见，与生活质量降低有关，并且仍然是囊性纤维化（CF）患者最常见的死亡原因。这些共识指南就个人达到 ACFLD 状态时出现的常见和独特问题的管理向 CF 社区提供了建议。方法 CF 基金会组建了一个多学科专家小组，由三个工作组组成：肺管理；合并症的管理；症状管理和社会心理问题。如果 ACFLD 中的管理考虑与整个 CF 人群中的管理考虑没有不同，或者已经在其他已发布的指南中解决，则排除主题。建议基于系统性文献回顾并在适当时结合专家意见。结果 委员会制定了 23 条针对 ACFLD 的推荐声明，涉及 ACFLD 的定义、肺部和重症监护病房管理、选定合并症的管理、症状控制和社会心理问题。结论 这些建议旨在与先前发布的针对整体 CF 人群的管理指南相结合，目的是减少 ACFLD 患者的实践差异并改善整体护理、生活质量和生存率。