Open AccessLumacaftor/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients
Author(s) -
Katherine Kutney,
Shan Donnola,
Chris A. Flask,
Rose Gubitosi-Klug,
MaryAnn O’Riordan,
Kimberly McBennett,
Thomas J. Sferra,
Beth Kaminski
Publication year - 2019
Publication title -
world journal of hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.913
H-Index - 55
ISSN - 1948-5182
DOI - 10.4254/wjh.v11.i12.761
Subject(s) - ivacaftor , medicine , steatosis , cystic fibrosis related diabetes , cystic fibrosis , cystic fibrosis transmembrane conductance regulator , diabetes mellitus , gastroenterology , insulin resistance , nonalcoholic fatty liver disease , population , body mass index , liver disease , fatty liver , endocrinology , impaired glucose tolerance , disease , environmental health
Hepatic steatosis is a common form of cystic fibrosis associated liver disease (CFLD) seen in an estimated 15%-60% of patients with cystic fibrosis (CF). The pathophysiology and health implications of hepatic steatosis in cystic fibrosis remain largely unknown. In the general population, hepatic steatosis is strongly associated with insulin resistance and type 2 diabetes. Cystic fibrosis related diabetes (CFRD) impacts 40%-50% of CF adults and is characterized by both insulin insufficiency and insulin resistance. We hypothesized that patients with CFRD would have higher levels of hepatic steatosis than cystic fibrosis patients without diabetes.