{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T08:20:18Z","timestamp":1788942018965,"version":"build-2803163510"},"reference-count":47,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2022,10,18]],"date-time":"2022-10-18T00:00:00Z","timestamp":1666051200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Hepatobiliary involvement is a hallmark in cystic fibrosis (CF), as the causative CF Transmembrane Conductance Regulator (CFTR) defect is expressed in the biliary tree. However, bile acid (BA) compositions in regard to pancreatic insufficiency, which is present at an early stage in about 85% of CF patients, have not been satisfactorily understood. We assess the pattern of serum BAs in people with CF (pwCF) without CFTR modulator therapy in regard to pancreatic insufficiency and the CFTR genotype. In 47 pwCF, 10 free and 12 taurine- and glycine-conjugated BAs in serum were prospectively assessed. Findings were related to genotype, pancreatic insufficiency prevalence (PIP)-score, and hepatic involvement indicated by serum liver enzymes, as well as clinical and ultrasound criteria for CF-related liver disease. Serum concentrations of total primary BAs and free cholic acid (CA) were significantly higher in pwCF with higher PIP-scores (p = 0.025, p = 0.009, respectively). Higher total BAs were seen in pwCF with PIP-scores \u22650.88 (p = 0.033) and with pancreatic insufficiency (p = 0.034). Free CA was higher in patients with CF-related liver involvement without cirrhosis, compared to pwCF without liver disease (2.3-fold, p = 0.036). pwCF with severe CFTR genotypes, as assessed by the PIP-score, reveals more toxic BA compositions in serum. Subsequent studies assessing changes in BA homeostasis during new highly effective CFTR-modulating therapies are of high interest.<\/jats:p>","DOI":"10.3390\/ijms232012436","type":"journal-article","created":{"date-parts":[[2022,10,18]],"date-time":"2022-10-18T00:31:01Z","timestamp":1666053061000},"page":"12436","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency"],"prefix":"10.3390","volume":"23","author":[{"given":"Harold","family":"Tabori","sequence":"first","affiliation":[{"name":"Cystic Fibrosis Center, Brandenburg Medical School (MHB) University, Klinikum Westbrandenburg, 14770 Brandenburg an der Havel, Germany"},{"name":"Internal Medicine, Alexianer Hedwigsh\u00f6he Hospital, 12526 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Schneider","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Centre, Jena University Hospital, 07740 Jena, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"L\u00fcth","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, 14770 Brandenburg an der Havel, Germany"},{"name":"Faculty of Health Sciences, Joint Faculty of the Brandenburg University of Technology Cottbus-Senftenberg, Brandenburg Medical School Theodor Fontane, University of Potsdam, 14469 Potsdam, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Zagoya","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Brandenburg Medical School (MHB) University, Klinikum Westbrandenburg, 14770 Brandenburg an der Havel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anton","family":"Barucha","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Brandenburg Medical School (MHB) University, Klinikum Westbrandenburg, 14770 Brandenburg an der Havel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Lehmann","sequence":"additional","affiliation":[{"name":"Institute of Medical Statistics, Jena University Hospital, 07743 Jena, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eberhard","family":"Kauf","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Centre, Jena University Hospital, 07740 Jena, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"Barth","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Pharmakologie und Toxikologie, Jena University Hospital, 07743 Jena, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen G.","family":"Mainz","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Brandenburg Medical School (MHB) University, Klinikum Westbrandenburg, 14770 Brandenburg an der Havel, Germany"},{"name":"Cystic Fibrosis Centre, Jena University Hospital, 07740 Jena, Germany"},{"name":"Faculty of Health Sciences, Joint Faculty of the Brandenburg University of Technology Cottbus-Senftenberg, Brandenburg Medical School Theodor Fontane, University of Potsdam, 14469 Potsdam, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1056\/NEJMe058293","article-title":"Restoring airway surface liquid in cystic fibrosis","volume":"354","author":"Ratjen","year":"2006","journal-title":"N. 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