Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors

Sabina Więcek; Halina Woś; Urszula Grzybowska-Chlebowczyk

doi:10.5114/pg.2013.38730

eISSN: 1897-4317
ISSN: 1895-5770

Gastroenterology Review/Przegląd Gastroenterologiczny

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5/2013
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abstract:

Review paper

Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors

Sabina Więcek

,

Halina Woś

,

Urszula Grzybowska-Chlebowczyk

Prz Gastroenterol 2013; 8 (5): 290–294

DOI: https://doi.org/10.5114/pg.2013.38730

Online publish date: 2013/10/28

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AMA

Więcek S, Woś H, Grzybowska-Chlebowczyk U. Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors. Gastroenterology Review/Przegląd Gastroenterologiczny. 2013;8(5):290-294. doi:10.5114/pg.2013.38730.

APA

Więcek, S., Woś, H.,  & Grzybowska-Chlebowczyk, U. (2013). Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors. Gastroenterology Review/Przegląd Gastroenterologiczny, 8(5), 290-294. https://doi.org/10.5114/pg.2013.38730

Chicago

Więcek, Sabina, Halina Woś,  and Urszula Grzybowska-Chlebowczyk. 2013. "Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors". Gastroenterology Review/Przegląd Gastroenterologiczny 8 (5): 290-294. doi:10.5114/pg.2013.38730.

Harvard

Więcek, S., Woś, H.,  and Grzybowska-Chlebowczyk, U. (2013). Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors. Gastroenterology Review/Przegląd Gastroenterologiczny, 8(5), pp.290-294. https://doi.org/10.5114/pg.2013.38730

MLA

Więcek, Sabina et al. "Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors." Gastroenterology Review/Przegląd Gastroenterologiczny, vol. 8, no. 5, 2013, pp. 290-294. doi:10.5114/pg.2013.38730.

Vancouver

Więcek S, Woś H, Grzybowska-Chlebowczyk U. Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors. Gastroenterology Review/Przegląd Gastroenterologiczny. 2013;8(5):290-294. doi:10.5114/pg.2013.38730.

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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.

keywords:

Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors

Sabina Więcek , Halina Woś , Urszula Grzybowska-Chlebowczyk

liver changes, cystic fibrosis, children

Sabina Więcek

,

Halina Woś

,

Urszula Grzybowska-Chlebowczyk