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Review
. 2018 May;34(3):146-151.
doi: 10.1097/MOG.0000000000000432.

Liver disease in patients with cystic fibrosis

Natasha Kamal  1 , Pallavi Surana  2 , Christopher Koh  2
Affiliations
Review

Liver disease in patients with cystic fibrosis

Natasha Kamal et al. Curr Opin Gastroenterol. 2018 May.

Abstract

Purpose of review: The aim of this study was to provide an overview of the current understanding of the pathophysiology, diagnosis and management of cystic fibrosis-liver disease (CFLD).

Recent findings: CFLD has a variety of manifestations. Previously, it was thought that patients progressed from mild cholestatic disease to cirrhosis to decompensated cirrhosis with portal hypertension. Newer evidence suggests that some patients may develop cirrhosis while others develop noncirrhotic portal hypertension. Advances in our understanding of the pathophysiology of disease necessitate modifications to the current diagnostic criteria. Both fibroscan and noninvasive biomarkers can be used to identify patients with cirrhosis and portal hypertension. Ursodeoxycholic acid remains the mainstay of therapy despite a paucity of rigorous studies supporting its use. Novel therapeutic agents such as CF transmembrane conductance regulator (CFTR) modulators and potentiators are encouraging but need to be evaluated specifically in CFLD.

Summary: A better understanding of the pathophysiology of disease is critical to developing more disease-specific diagnostics and therapeutics.

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Conflict of interest statement

Conflicts of interest:

None of the authors have financial interests or conflicts of interest related to this research.

Figures

Figure 1
Figure 1
Cholangiolar cholestatsis with bile plugs (long arrow) and granular debris (arrowhead) (H&E, 600x). (B) Focal biliary cirrhosis (elsewhere on the section of the architecture was intact) (Masson trichrome, 40x) (C) Portal venous occlusion with dense fibrosis (arrowhead) (Masson Trichrome, 100x). (D) Nodularity seen in region with the appearance of nodular regenerative hyperplasia (arrowheads outline nodule) (Masson trichrome, 40x). (Images courtesy of Dr. David Kleiner).
See this image and copyright information in PMC

References

    1. Farrell PM, Rosenstein BJ, White TB, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008;153(2):S4–S14. - PMC - PubMed
    1. Sontag MK, Hammond KB, Zielenski J, et al. Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes. J Pediatr. 2005;147(3 Suppl):S83–8. - PubMed
    1. Cohn JA, Strong TV, Picciotto MR, et al. Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology. 1993;105(6):1857–64. - PubMed
    1. Leung DH, Narkewicz MR. Cystic Fibrosis-related cirrhosis. J Cyst Fibros. 2017. - PubMed
    1. Hillaire S, Cazals-Hatem D, Bruno O, et al. Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy. Liver Transpl. 2017;23(10):1342–7. - PubMed

MeSH terms