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

ABSTRACT: 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 testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with newly proposed CFLD criteria. Thirty-six CF patients were followed for a median of 24.5 years (interquartile range 15.6-32.9). By the last follow-up, 11 (31%) had died. With conventional criteria, 8 (22%) patients had CFLD; and by the new criteria, 17 (47%) had CFLD at a median age of 36.6 years (interquartile range 26.5-43.2). By the new criteria, those with CFLD had higher median alanine amin

SUBMITTER: Koh C 

PROVIDER: S-EPMC5519421 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature