Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis.

ABSTRACT:

Background & aims

Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been characterized fully.

Methods

We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013.

Results

Male patients were diagnosed with liver

SUBMITTER: Stonebraker JR 

PROVIDER: S-EPMC4955685 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature