Format

Send to

Choose Destination
See comment in PubMed Commons below
J Clin Gastroenterol. 2009 Oct;43(9):858-64. doi: 10.1097/MCG.0b013e31819e8bbd.

Hepatobiliary abnormalities and disease in cystic fibrosis: epidemiology and outcomes through adulthood.

Author information

1
Division of Gastroenterology/Hepatology, Indiana University Medical Center, Indianapolis, Indiana, USA.

Abstract

OBJECTIVES:

There is limited data regarding the prevalence of hepatobiliary disease in North American patients with cystic fibrosis (CF) through adulthood. Our aim was to determine the prevalence of, and risk factors for, CF-related hepatobiliary abnormalities and determine factors that predict the development of CF-related hepatobiliary disease.

METHODS:

We performed a retrospective cohort study of all CF patients who presented to a UnitedStates tertiary care referral academic center over a 32-year period. "CF-related hepatobiliary abnormality" was defined as the presence of abnormal liver chemistries on one or more occasion and "CF-related hepatobiliary disease" was defined as biochemical, physical examination, or ultrasonographic abnormalities on at least 2 consecutive examinations spanning a 1-year period.

RESULTS:

Two-hundred eighty-three CF patients who presented between the years 1970 and 2002 were identified, with an age range of 2 months to 63 years. Sixty-five percent had CF-related hepatobiliary abnormalities with a higher prevalence seen in CF patients <18 years of age (84% vs. 16%, P<0.01). Fifteen percent of our cohort had CF-related hepatobiliary disease with 93% of cases occurring in individuals before age 18. One quarter of individuals with CF-related hepatobiliary abnormalities developed hepatobiliary disease.

CONCLUSIONS:

Abnormal liver chemistries in CF are common though most of CF patients lack clinical evidence of liver disease and the severe complications of fibrosis/cirrhosis are rare. The risk of liver involvement decreases significantly with age, falling by 10% per annum for those described as having CF-related hepatobiliary disease. CF-related hepatobiliary disease is a rare occurrence after age 18.

PMID:
19525864
DOI:
10.1097/MCG.0b013e31819e8bbd
[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Lippincott Williams & Wilkins
    Loading ...
    Support Center