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Hepatic manifestations of cystic fibrosis

机译：肝纤维化的肝脏表现

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Purpose of review Liver disease in cystic fibrosis (CF) usually develops before puberty, is often asymptomatic and slowly progressive. Multilobular cirrhosis develops in approximately 5-10% of patients by the age of 18, and is a significant contributor to the morbidity and mortality. No therapy, including ursodeoxycholic acid and cystic fibrosis transmembrane conductance regulator correctors or potentiators, has proven effective to prevent or halt the progression of liver disease towards cirrhosis and portal hypertension. This review provides the current knowledge in the epidemiology of CF liver disease and development of noninvasive tools to assess liver disease severity and progression overtime in order to optimize clinical management and therapeutic options. Recent findings Liver disease not only develops during childhood but also later in the lifetime of patients with CF; the incidence of cirrhosis with portal hypertension increases progressively reaching 10% by age 30. Several noninvasive tools to measure liver stiffness as an indirect measure of fibrosis are being investigated, and show promising results for the assessment of early stages of liver fibrosis and disease progression. Identifying noninvasive biomarkers is fundamental to improving early diagnosis, monitoring disease evolution and measuring treatment effects. A prerequisite is the use of consistent definitions for CF- liver disease (LD) in clinical trials.
机译：审查肝脏疾病在囊性纤维化（CF）通常在青春期之前发育，通常是无症状和慢慢进行的。多发性肝硬化在18岁时，大约5-10％的患者发展，是发病率和死亡率的重要贡献者。没有治疗，包括熊酸胆酸和囊性纤维化跨膜电导调节器校正器或增强剂，已被证明有效预防或停止肝脏病的进展，以肝硬化和门静脉高血压。本综述提供了CF肝病流行病学的现有知识，以及不诱导工具的开发，以评估肝病严重程度和进展加班，以优化临床管理和治疗选择。最近的发现肝病不仅在儿童时期发展，而且在CF患者的寿命之后也是如此。随着30岁的30％，肝硬化的发病率逐渐增加了10％。正在研究以肝硬化作为间接纤维化的间接测量来测量肝硬化的几种非侵入性工具，并表现出对肝纤维化和疾病进展的早期阶段的有希望的结果。鉴定非侵入性生物标志物是改善早期诊断，监测疾病演化和测量治疗效果的基础。先决条件是在临床试验中使用CF-Liver疾病（LD）的一致定义。

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