Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity

• Published in: Hepatology (Baltimore, Md.) Vol. 66; no. 2; pp. 591 - 601
• Main Authors: Koh, Christopher, Sakiani, Sasan, Surana, Pallavi, Zhao, Xiongce, Eccleston, Jason, Kleiner, David E., Herion, David, Liang, T. Jake, Hoofnagle, Jay H., Chernick, Milica, Heller, Theo
• Format: Journal Article
• Language: English
• Published: United States Wolters Kluwer Health, Inc 01.08.2017
• Subjects: Adult; Age of Onset; Alanine; Alanine transaminase; Aspartate aminotransferase; Bilirubin; Biopsy, Needle; Cause of Death; Child; Child, Preschool; Children; Cohort Studies; Cystic fibrosis; Cystic Fibrosis - diagnosis; Cystic Fibrosis - epidemiology; Diagnosis; Female; Hepatology; Humans; Hyperplasia; Immunohistochemistry; Liver diseases; Liver Diseases - diagnosis; Liver Diseases - epidemiology; Liver Function Tests; Male; Morbidity; Prospective Studies; Prothrombin; Risk Assessment; Severity of Illness Index; Survival Analysis

Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations. Adult‐onset CFLD lacks sufficient characterization and diagnostic tools. A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic test...