Hepatic Manifestations of Cystic Fibrosis

Overview

Journal Clin Liver Dis

Specialty Gastroenterology

Date 2019 Apr 6

PMID 30947876

Citations 15

Authors

Sasan Sakiani

David E Kleiner

Theo Heller

Christopher Koh

Affiliations

Soon will be listed here.

Abstract

Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant.

Citing Articles

Sotagliflozin attenuates liver-associated disorders in cystic fibrosis rabbits.

Liang X, Hou X, Bouhamdan M, Sun Y, Song Z, Rajagopalan C JCI Insight. 2024; 9(6).

PMID: 38358827 PMC: 10972622. DOI: 10.1172/jci.insight.165826.

Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children.

Tan A, Demirtas D, Hizarcioglu-Gulsen H, Karakaya J, Isiyel E, Ozen H Pediatr Radiol. 2023; 54(2):250-259.

PMID: 38133654 DOI: 10.1007/s00247-023-05832-1.

Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations.

Sellers Z, Assis D, Paranjape S, Sathe M, Bodewes F, Bowen M Hepatology. 2023; 79(5):1220-1238.

PMID: 37934656 PMC: 11020118. DOI: 10.1097/HEP.0000000000000646.

Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes.

Wu Q, Liang X, Hou X, Song Z, Bouhamdan M, Qiu Y PNAS Nexus. 2023; 2(1):pgac306.

PMID: 36712930 PMC: 9832953. DOI: 10.1093/pnasnexus/pgac306.

Fatty liver disease that is neither metabolic nor alcoholic.

Allende D, Kleiner D Hum Pathol. 2023; 141:212-221.

PMID: 36702356 PMC: 10363575. DOI: 10.1016/j.humpath.2023.01.003.

References

Colombo C, Alicandro G . Liver Disease in Cystic Fibrosis: Illuminating the Black Box. Hepatology. 2018; 69(4):1379-1381. DOI: 10.1002/hep.30255. View

Dowman J, WATSON D, Loganathan S, Gunson B, Hodson J, Mirza D . Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis. Am J Transplant. 2012; 12(4):954-64. DOI: 10.1111/j.1600-6143.2011.03904.x. View

Mendizabal M, Reddy K, Cassuto J, Olthoff K, Faust T, Makar G . Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data. Liver Transpl. 2011; 17(3):243-50. DOI: 10.1002/lt.22240. View

Boelle P, Debray D, Guillot L, Clement A, Corvol H . Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients. Hepatology. 2018; 69(4):1648-1656. PMC: 6519059. DOI: 10.1002/hep.30148. View

Leung D, Khan M, Minard C, Guffey D, Ramm L, Clouston A . Aspartate aminotransferase to platelet ratio and fibrosis-4 as biomarkers in biopsy-validated pediatric cystic fibrosis liver disease. Hepatology. 2015; 62(5):1576-83. DOI: 10.1002/hep.28016. View

Lamireau T, Monnereau S, Martin S, Marcotte J, Winnock M, Alvarez F . Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol. 2004; 41(6):920-5. DOI: 10.1016/j.jhep.2004.08.006. View

Poetter-Lang S, Staufer K, Baltzer P, Tamandl D, Muin D, Bastati N . The Efficacy of MRI in the diagnostic workup of cystic fibrosis-associated liver disease: A clinical observational cohort study. Eur Radiol. 2018; 29(2):1048-1058. PMC: 6302923. DOI: 10.1007/s00330-018-5650-5. View

van der Feen C, van der Doef H, van der Ent C, Houwen R . Ursodeoxycholic acid treatment is associated with improvement of liver stiffness in cystic fibrosis patients. J Cyst Fibros. 2016; 15(6):834-838. DOI: 10.1016/j.jcf.2016.07.009. View

Ramsey B, Davies J, McElvaney N, Tullis E, Bell S, Drevinek P . A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011; 365(18):1663-72. PMC: 3230303. DOI: 10.1056/NEJMoa1105185. View

10.

Flass T, Narkewicz M . Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros. 2012; 12(2):116-24. PMC: 3883947. DOI: 10.1016/j.jcf.2012.11.010. View

11.

Werlin S, Scotet V, Uguen K, Audrezet M, Cohen M, Yaakov Y . Primary sclerosing cholangitis is associated with abnormalities in CFTR. J Cyst Fibros. 2018; 17(5):666-671. DOI: 10.1016/j.jcf.2018.04.005. View

12.

Bolia R, Ooi C, Lewindon P, Bishop J, Ranganathan S, Harrison J . Practical approach to the gastrointestinal manifestations of cystic fibrosis. J Paediatr Child Health. 2018; 54(6):609-619. DOI: 10.1111/jpc.13921. View

13.

Lindblad A, Glaumann H, Strandvik B . Natural history of liver disease in cystic fibrosis. Hepatology. 1999; 30(5):1151-8. DOI: 10.1002/hep.510300527. View

14.

Kamal N, Surana P, Koh C . Liver disease in patients with cystic fibrosis. Curr Opin Gastroenterol. 2018; 34(3):146-151. PMC: 7232742. DOI: 10.1097/MOG.0000000000000432. View

15.

Gominon A, Frison E, Hiriart J, Vergniol J, Clouzeau H, Enaud R . Assessment of Liver Disease Progression in Cystic Fibrosis Using Transient Elastography. J Pediatr Gastroenterol Nutr. 2017; 66(3):455-460. DOI: 10.1097/MPG.0000000000001822. View

16.

Arnon R, Annunziato R, Miloh T, Padilla M, Sogawa H, Batemarco L . Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the UNOS database. Pediatr Transplant. 2011; 15(3):254-64. DOI: 10.1111/j.1399-3046.2010.01460.x. View

17.

Jeanniard-Malet O, Duche M, Fabre A . Survey on Clinical Practice of Primary Prophylaxis in Portal Hypertension in Children. J Pediatr Gastroenterol Nutr. 2016; 64(4):524-527. DOI: 10.1097/MPG.0000000000001453. View

18.

Spirli C, Granato A, Zsembery K, Anglani F, Okolicsanyi L, Larusso N . Functional polarity of Na+/H+ and Cl-/HCO3- exchangers in a rat cholangiocyte cell line. Am J Physiol. 1998; 275(6):G1236-45. DOI: 10.1152/ajpgi.1998.275.6.G1236. View

19.

Davison S . Assessment of liver disease in cystic fibrosis. Paediatr Respir Rev. 2018; 27:24-27. DOI: 10.1016/j.prrv.2018.05.010. View

20.

Mc Ewan F, Hodson M, Simmonds N . The prevalence of "risky behaviour" in adults with cystic fibrosis. J Cyst Fibros. 2011; 11(1):56-8. DOI: 10.1016/j.jcf.2011.09.002. View