Więcek Sabina, Woś Halina, Grzybowska-Chlebowczyk Urszula
Department of Paediatrics, Medical University of Silesia, Katowice, Poland.
Prz Gastroenterol. 2013;8(5):290-4. doi: 10.5114/pg.2013.38730. Epub 2013 Oct 28.
Liver changes observed in the course of cystic fibrosis comprise a group of complex processes of fibrosis, inflammation, remodelling, apoptosis and cholestasis as a result of abnormal functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, immunological reactions and response to oxidation stress. Liver lesions are only observed in 5-20% of patients with diagnosed cystic fibrosis; however, they increase mortality, reduce the lifespan and deteriorate the quality of life. Liver diseases are the most common extrapulmonary causes of death in patients with cystic fibrosis. The aim of the study was to analyse the hitherto performed studies on the aetiopathogenesis of liver changes in the course of cystic fibrosis considering disturbances of the bile acid profile as well as genetic and immunological factors.
在囊性纤维化病程中观察到的肝脏变化包括一组复杂的纤维化、炎症、重塑、细胞凋亡和胆汁淤积过程，这些过程是由囊性纤维化跨膜传导调节因子（CFTR）蛋白功能异常、免疫反应和对氧化应激的反应引起的。肝脏病变仅在5%-20%的已确诊囊性纤维化患者中观察到；然而，它们会增加死亡率、缩短寿命并降低生活质量。肝脏疾病是囊性纤维化患者最常见的肺外死亡原因。本研究的目的是分析迄今为止进行的关于囊性纤维化病程中肝脏变化的病因发病机制的研究，同时考虑胆汁酸谱的紊乱以及遗传和免疫因素。
