Division of Gastroenterology and Hepatology, Manchester University NHS Foundation Trust, Manchester, UK.
Faculty of Biology Medicine and Health, The University of Manchester, Manchester, UK.
Aliment Pharmacol Ther. 2022 Feb;55(4):389-400. doi: 10.1111/apt.16749. Epub 2022 Jan 19.
Cystic fibrosis-related liver disease (CFLD) is the leading cause of death in cystic fibrosis (CF), after pulmonary disease. To improve identification and management of this condition requires an understanding of the underlying disease mechanism.
This review summarises the current understanding of CFLD epidemiology, pathology, diagnosis and management.
Relevant reports on cystic fibrosis liver disease were identified using a literature search and summarised.
CFLD is a heterogeneous condition with several different co-existent pathologies, including environmental and genetic factors. Incidence of clinically significant CFLD continues at a linear rate into early adulthood and has been described in up to 25% of CF patients. Diagnosis strategies lack precision and patient risk stratification needs to look beyond Childs-Pugh scoring. Efficacious therapies are lacking and, at present, newer modulator therapies lack data in CFLD and carry an increased risk of hepatotoxicity. Outcomes of liver transplant are comparable to non-CF transplant indications.
The incidence of CFLD increases with age and hence is increasingly important to adult patients with CF. Effective therapies are lacking. For progress to be made a better understanding of pathogenesis and disease detection are required.
囊性纤维化相关肝病（CFLD）是囊性纤维化（CF）继肺部疾病之后导致死亡的主要原因。为了改善对此类疾病的识别和管理，需要了解其潜在的发病机制。
本文总结了囊性纤维化肝病的流行病学、病理学、诊断和管理的现有认识。
使用文献检索，确定并总结了有关囊性纤维化肝脏疾病的相关报告。
CFLD 是一种异质性疾病，存在几种不同的共存病理学，包括环境和遗传因素。临床上明显的 CFLD 的发生率以线性速率持续到成年早期，在多达 25%的 CF 患者中均有描述。诊断策略缺乏准确性，患者风险分层需要超越 Childs-Pugh 评分。缺乏有效的治疗方法，目前，新型调节剂治疗方法在 CFLD 中的数据不足，且具有更高的肝毒性风险。肝移植的结局与非 CF 移植适应证相当。
CFLD 的发病率随年龄增长而增加，因此对 CF 成年患者变得愈发重要。目前缺乏有效的治疗方法。为了取得进展，需要更好地了解发病机制和疾病检测。
