PDF(Sci-hub)
Abstract：
Liver disease affects one-third of patients with cystic fibrosis (CF) and it is one of the major causes of morbidity and mortality in these patients. Historically considered a disease of childhood, its impact is now seen more often in adulthood. The heterogeneous pattern of CF liver disease and its rapid progression to cirrhosis remain a diagnostic challenge and new questions pertaining to the nature of liver involvement have recently been raised. Non-invasive measures to stratify the severity of liver involvement are increasingly used to predict clinical outcomes. A single treatment, ursodeoxycholic acid, has been used to slow progression of liver disease while recent advances in the field of CF treatments are promising. Management of portal hypertension remains challenging but outcomes after liver transplantation are encouraging. While many questions remain unanswered, a growing number of CF patients reach adulthood and will require care for CF liver disease.
摘要：
肝病影响三分之一的囊性纤维化（CF）患者，是这些患者发病和死亡的主要原因之一。历史上被认为是一种儿童疾病，它的影响现在更常见于成年期。CF肝病的异质性模式及其快速进展为肝硬化仍然是诊断挑战，最近提出了有关肝脏受累性质的新问题。对肝脏受累的严重程度进行分层的非侵入性措施越来越多地用于预测临床结果。单一治疗，熊去氧胆酸,已经被用于减缓肝病的进展,而CF治疗领域的最新进展是有希望的。门静脉高压的治疗仍然具有挑战性，但肝移植后的结果令人鼓舞。虽然许多问题仍然没有答案，越来越多的CF患者成年后需要治疗CF肝病.
