eISSN: 1897-4317
ISSN: 1895-5770
Gastroenterology Review/Przegląd Gastroenterologiczny
Current issue Archive Manuscripts accepted About the journal Abstracting and indexing Subscription Contact Instructions for authors
SCImago Journal & Country Rank
vol. 9
Review paper

Liver disease in cystic fibrosis

Natalia Kobelska-Dubiel, Beata Klincewicz, Wojciech Cichy

Prz Gastroenterol 2014; 9 (3): 136–141
Online publish date: 2014/06/26
View full text
Get citation
JabRef, Mendeley
Papers, Reference Manager, RefWorks, Zotero
Cystic fibrosis-associated liver disease (CFLD) affects ca. 30% of patients. The CFLD is now considered the third cause of death, after lung disease and transplantation complications, in CF patients. Diagnostics, clinical assessment and treatment of CFLD have become a real challenge since a striking increase of life expectancy in CF patients has recently been observed. There is no elaborated “gold standard” in the diagnostic process of CFLD; clinical evaluation, laboratory tests, ultrasonography and liver biopsy are used. Clinical forms of CFLD are elevation of serum liver enzymes, hepatic steatosis, focal biliary cirrhosis, multilobular biliary cirrhosis, neonatal cholestasis, cholelithiasis, cholecystitis and micro-gallbladder. In children, CFLD symptoms mostly occur in puberty. Clinical symptoms appear late, when damage of the hepatobiliary system is already advanced. The CFLD is more common in patients with severe mutations of CFTR gene, in whom a complete loss of CFTR protein function is observed. CFLD, together with exocrine pancreatic insufficiency and meconium ileus, is considered a component of the severe CF phenotype. Treatment of CFLD should be complex and conducted by a multispecialist team (gastroenterologist, hepatologist, dietician, radiologist, surgeon). The main aim of the treatment is to prevent liver damage and complications associated with portal hypertension and liver cirrhosis. Ursodeoxycholic acid is used in the treatment of CFLD. There is no treatment of proven long-term efficacy in CFLD. Liver transplantation is a treatment of choice in end-stage liver disease.

cystic fibrosis, liver disease, diagnostics, treatment


Siano M, De Gregorio F, Boggia B, et al. Ursodeoxycholic acid treatment in patients with cystic fibrosis at risk for liver disease. Dig Liver Dis 2010; 42: 428-31.

Diwakar V, Pearson L, Beath S. Liver disease in children with cystic fibrosis. Paediatr Respir Rev 2001; 2: 340-9.

Herrmann U, Dockter G, Lammert F. Cystic fibrosis-associated liver disease. Best Pract Res Clin Gastroenterol 2010; 24: 585-92.

Minagawa N, Nagata J, Shibao K, et al. Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile. Gastroenterology 2007; 133: 1592-602.

Fiorotto R, Spirlì C, Fabris L, et al. Ursodeoxycholic acid stimulates cholangiocyte fluid secretion in mice via CFTR-dependent ATP secretion. Gastroenterology 2007; 133: 1603-13.

Majka L, Pogorzelski A, Młynarczyk W, et al. Effect of genotype on selected clinical features of Polish cystic fibrosis adults. J Appl Genet 2001; 42: 367-77.

McKone EF, Emerson SS, Edwards KL, et al. Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet 2003; 361: 1671-6.

Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors and outcome. Hepatology 2002; 36: 1374-82.

Lindblad A, Glaumann H, Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology 1999; 30: 1151-8.

Shapira R, Hadzic N, Francavilla R, et al. Retrospective review of cystic fibrosis presenting as infantile liver disease. Arch Dis Child 1999; 81: 125-8.

Corbett K, Kelleher S, Rowland M, et al. Cystic fibrosis-associated liver disease: a population-based study. J Pediatr 2004; 145: 327-32.

Colombo C, Crosignani A, Battezzati PM. Liver involvement in cystic fibrosis. J Hepatol 1999; 31: 946-54.

Littlewood JM. Good care for people with cystic fibrosis. Paediatr Respir Rev 2000; 1: 179-89.

Lamireau T, Monnereau S, Martin S, et al. Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 2004; 41: 920-5.

Wilschanski M, Miller LL, Shoseyov D, et al. Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis. Eur Respir J 2011; 38: 59-69.
Quick links
© 2018 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.
PayU - płatności internetowe