Cystic Fibrosis-related cirrhosis

Daniel H Leung; Michael R Narkewicz

doi:10.1016/j.jcf.2017.07.002

Cystic Fibrosis-related cirrhosis

J Cyst Fibros. 2017 Nov:16 Suppl 2:S50-S61. doi: 10.1016/j.jcf.2017.07.002.

Authors

Daniel H Leung¹, Michael R Narkewicz²

Affiliations

¹ Division of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin St, Houston, TX 77030, United States. Electronic address: [email protected].
² Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado SOM, Digestive Health Institute, Children's Hospital Colorado, 13123 East 16th Ave, Aurora, CO 80045, United States. Electronic address: [email protected].

PMID: 28986027
DOI: 10.1016/j.jcf.2017.07.002

Abstract

While liver involvement is common in cystic fibrosis, the major liver disorder with impact on the clinical outcome of individuals with CF is the development of multilobular cirrhosis with progression to portal hypertension. Interestingly, this is a disorder primarily of children and adolescents. We review the proposed pathogenesis, clinical presentation, diagnostic work-up, medical and surgical management, and complications of CF cirrhosis.

Keywords: Cirrhosis; Liver disease; Portal hypertension.

Publication types

Review

MeSH terms

Cystic Fibrosis / complications*
Disease Management
Disease Progression
Humans
Hypertension, Portal* / diagnosis
Hypertension, Portal* / etiology
Liver Cirrhosis* / diagnosis
Liver Cirrhosis* / etiology
Liver Cirrhosis* / physiopathology
Liver Cirrhosis* / therapy