Abstract：
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.
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.
摘要：
囊性纤维化相关性肝病（CFLD）是囊性纤维化（CF）的主要死亡原因，肺病后。为了改善这种状况的识别和管理，需要了解潜在的疾病机制。
这篇综述总结了目前对CFLD流行病学的理解，病理学,诊断和管理。
使用文献检索并总结了囊性纤维化肝病的相关报道。
CFLD是一种具有几种不同共存病理的异质病症，包括环境和遗传因素。临床上显着的CFLD的发生率以线性速率持续到成年早期，并且已经在高达25％的CF患者中进行了描述。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,较新的调质疗法缺乏CFLD数据,肝毒性风险增加.肝移植的结果与非CF移植适应症相当。
CFLD的发生率随着年龄的增长而增加，因此对患有CF的成年患者越来越重要。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理和疾病检测。
这篇综述总结了目前对CFLD流行病学的理解，病理学,诊断和管理。
使用文献检索并总结了囊性纤维化肝病的相关报道。
CFLD是一种具有几种不同共存病理的异质病症，包括环境和遗传因素。临床上显着的CFLD的发生率以线性速率持续到成年早期，并且已经在高达25％的CF患者中进行了描述。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,较新的调质疗法缺乏CFLD数据,肝毒性风险增加.肝移植的结果与非CF移植适应症相当。
CFLD的发生率随着年龄的增长而增加，因此对患有CF的成年患者越来越重要。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理和疾病检测。
