Published online Jan 27, 2018. doi: 10.4254/wjh.v10.i1.34
Peer-review started: October 24, 2017
First decision: November 23, 2017
Revised: December 6, 2017
Accepted: December 13, 2017
Article in press: December 13, 2017
Published online: January 27, 2018
To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis (CF) patients with hepatic steatosis as compared to normal CF controls.
We performed a retrospective review of adult CF patients in an academic outpatient setting during 2016. Baseline characteristics, genetic mutation analysis as well as laboratory values were collected. Abdominal imaging (ultrasound, computed tomography, magnetic resonance) was used to determine presence of hepatic steatosis. We compare patients with hepatic steatosis to normal controls.
Data was collected on 114 patients meeting inclusion criteria. Seventeen patients (14.9%) were found to have hepatic steatosis on imaging. Being overweight (BMI > 25) (P = 0.019) and having a higher ppFEV1 (75 vs 53, P = 0.037) were significantly associated with hepatic steatosis. Patients with hepatic steatosis had a significantly higher median alanine aminotransferase level (27 vs 19, P = 0.048). None of the hepatic steatosis patients had frank CF liver disease, cirrhosis or portal hypertension. We found no significant association with pancreatic insufficiency or CF related diabetes.
Hepatic steatosis appears to be a clinically and phenotypically distinct entity from CF liver disease. The lack of association with malnourishment and the significant association with higher BMI and higher ppFEV1 demonstrate similarities with non-alcoholic fatty liver disease. Long term prospective studies are needed to ascertain whether CF hepatic steatosis progresses to fibrosis and cirrhosis.
Core tip: Our retrospective cohort study of cystic fibrosis (CF) patients with hepatic steatosis demonstrates that hepatic steatosis in CF is associated with a higher body mass index as well as a higher percent predicted forced expiratory volume in 1 s, as compared to normal CF controls. None of our patients with hepatic steatosis exhibited evidence for advanced liver disease. Our findings are novel and demonstrate similarities between hepatic steatosis in CF and adult non-alcoholic fatty liver disease and future prospective studies are required to determine whether this steatosis may evolve into cirrhosis.