Department of Pathology, Baylor College of Medicine, Houston, Texas.
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
Clin Gastroenterol Hepatol. 2019 Sep;17(10):2134-2136. doi: 10.1016/j.cgh.2018.10.046. Epub 2018 Nov 4.
Cystic fibrosis liver disease (CFLD) has long been postulated to be secondary to dysfunctional cystic fibrosis transmembrane conductance regulator in the apical biliary epithelium, leading to bile stasis and eventually cirrhosis with portal hypertension. However, pathologic changes in the cystic fibrosis (CF) liver are distinct from the pancreas and lungs in that fibrocystic changes are absent. Furthermore, the lack of clinically evident biliary obstruction and liver dysfunction suggest there may be alternative mechanisms that contribute to CFLD. Two recent studies in young adults described obliterative portal venopathy (OPV) and noncirrhotic portal hypertension (NCPH) as the predominant pathophysiology in young adults (median, 22 y) with CFLD. It is unknown if OPV develops early in childhood. Herein, we report the clinical features and liver pathology in 17 explants from children and adolescents with CF, representing 13.6% (17 of 125) of the CF liver transplant population in the United States according to the United Network for Organ Sharing and Organ Procurement and Transplantation Network.
囊性纤维化肝病（CFLD）长期以来被认为是由于顶端胆管上皮中囊性纤维化跨膜电导调节因子功能障碍导致胆汁淤积，最终导致肝硬化和门静脉高压。然而，囊性纤维化（CF）肝脏的病理变化与胰腺和肺部不同，不存在纤维囊性变化。此外，缺乏明显的临床胆道梗阻和肝功能障碍表明，可能存在其他导致 CFLD 的机制。最近两项针对年轻成年人的研究描述了闭塞性门静脉病（OPV）和非肝硬化性门静脉高压（NCPH）是年轻成年人（中位年龄 22 岁）CFLD 的主要病理生理学。目前尚不清楚 OPV 是否在儿童期早期发生。在此，我们报告了美国器官共享联合网络和器官获取和移植网络中 17 名 CF 儿童和青少年肝移植供体的临床特征和肝脏病理学，占美国 CF 肝移植人群的 13.6%（17/125）。
