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      Modifier genes in cystic fibrosis-related liver disease.

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          Abstract

          Cystic fibrosis (CF; OMIM 219700) is caused by variations in the cystic fibrosis transmembrane conductance regulator gene. CF-related liver disease (CFLD) affects approximately one-third of patients with CF, but the severity of CFLD is highly variable. This review provides the latest knowledge in the pathophysiology and CF genetic modifier research in CFLD.

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          Author and article information

          Journal
          Curr. Opin. Gastroenterol.
          Current opinion in gastroenterology
          Ovid Technologies (Wolters Kluwer Health)
          1531-7056
          0267-1379
          Mar 2019
          : 35
          : 2
          Affiliations
          [1 ] INSERM, Centre de Recherche Saint-Antoine (CRSA), Sorbonne Université.
          [2 ] Pediatric Hepatology Unit, Reference Centre for Biliary Atresia and Genetic Cholestatic Diseases (CRMR, AVB-CG), Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP) - Université Paris V.
          [3 ] Pneumologie Pédiatrique, AP-HP, Hôpital Trousseau.
          [4 ] Department of Hepatology, Reference Centre for Inflammatory Biliary Diseases and Autoimmune Hepatitis (CRMR, MIVB-H), AP-HP, Hôpital Saint-Antoine, Paris, France.
          Article
          10.1097/MOG.0000000000000508
          30585791

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