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The causes of the heterogeneity of clinical manifestations, ranging from steatosis to focal biliary cholestasis and biliary strictures, are poorly understood and likely reflect a variety of environmental and disease\u2010modifying factors in the setting of underlying\n                    <jats:italic>CFTR<\/jats:italic>\n                    mutations. This review summarizes the current understanding of the pathophysiology of hepatobiliary manifestations of CF, and discusses emerging disease models and therapeutic approaches that hold promise to impact this important yet incompletely addressed aspect of CF care.\n                  <\/jats:p>","DOI":"10.1002\/ppul.26869","type":"journal-article","created":{"date-parts":[[2024,8,6]],"date-time":"2024-08-06T05:34:59Z","timestamp":1722922499000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Pathophysiology of Cystic Fibrosis Liver Disease"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9774-1478","authenticated-orcid":false,"given":"Vania L.","family":"Kasper","sequence":"first","affiliation":[{"name":"The Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Hasbro Children's Hospital Warren Alpert Medical School of Brown University Providence Rhode Island USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8941-6170","authenticated-orcid":false,"given":"David N.","family":"Assis","sequence":"additional","affiliation":[{"name":"Section of Digestive Diseases Yale School of Medicine New Haven Connecticut USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,6]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.30148"},{"key":"e_1_2_11_3_1","unstructured":"Cystic Fibrosis Foundation Patient Registry \u2010 2021 Annual Data Report. 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