Division of Gastroenterology and Hepatology, University of Maryland School of Medicine, University of Maryland Medical Center, 22 South Greene Street, N3W50, Baltimore, MD 21201, USA.
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, 10 Center Drive, Building 10, Room 2S235, MSC 1500, Bethesda, MD 20892, USA.
Clin Liver Dis. 2019 May;23(2):263-277. doi: 10.1016/j.cld.2018.12.008. Epub 2019 Feb 21.
Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant.
囊性纤维化肝病（CFLD）仍然是囊性纤维化患者的第三大死亡原因。尽管大多数 CFLD 患者在儿童期出现，但最近的研究表明，成年人中也存在第二波肝病。目前尚无明确的 CFLD 诊断指南。CFLD 的治疗选择仍然有限，UDCA 虽然被广泛应用，但长期疗效尚不清楚。那些出现肝功能失代偿或无法控制的静脉曲张出血的患者可能受益于单独或与肺移植联合进行肝移植。
