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

Cystic Fibrosis-related cirrhosis

Daniel H Leung  1 , Michael R Narkewicz  2
Affiliations
Free article
Review

Cystic Fibrosis-related cirrhosis

Daniel H Leung et al. J Cyst Fibros. 2017 Nov.
Free article

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.

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