Liver disease in cystic fibrosis

Lisette Leeuwen; Dominic A Fitzgerald; Kevin J Gaskin

doi:10.1016/j.prrv.2013.05.001

Liver disease in cystic fibrosis

Paediatr Respir Rev. 2014 Mar;15(1):69-74. doi: 10.1016/j.prrv.2013.05.001. Epub 2013 Jun 14.

Authors

Lisette Leeuwen¹, Dominic A Fitzgerald², Kevin J Gaskin³

Affiliations

¹ Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, Australia; Medical School, University of Groningen, Groningen, The Netherlands.
² Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia.
³ Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia; Department of Gastroenterology, The Children's Hospital at Westmead, Sydney, Australia; James Fairfax Institute of Paediatric Nutrition, University of Sydney, Sydney, Australia. Electronic address: [email protected].

PMID: 23769887
DOI: 10.1016/j.prrv.2013.05.001

Abstract

The survival of patients with cystic fibrosis (CF) has progressively increased over recent decades, largely attributable to early diagnosis through newborn screening and advances in nutritional and respiratory care. As the life expectancy of patients with CF has improved, non-respiratory complications such as liver disease have become increasingly recognized. Biochemical derangements of liver enzymes in CF are common and may be attributed to a number of specific hepatobiliary abnormalities. Among them, Cystic Fibrosis-associated Liver Disease (CFLD) is clinically the most significant hepatic complication and is believed to have a significant impact on morbidity and mortality. However, there remains much conjecture about the extent of the adverse prognostic implications that a diagnosis of CFLD has on clinical outcomes. The purpose of this review is to give an overview of the current knowledge regarding liver disease in children with CF.

Keywords: Cholestasis; Cystic Fibrosis; Cystic Fibrosis-associated Liver Disease; Liver transplantation; Morbidity; Mortality.

Publication types

Review

MeSH terms

Child
Cystic Fibrosis / complications*
Early Diagnosis*
Global Health
Humans
Incidence
Liver Diseases* / diagnosis
Liver Diseases* / epidemiology
Liver Diseases* / etiology
Prognosis
Risk Factors