Loss of ileum decreases serum fibroblast growth factor 19 in relation to liver inflammation and fibrosis in pediatric onset intestinal failure

Background & Aims The pathogenesis of intestinal failure (IF) associated liver disease (IFALD) is uncertain, we therefore investigated the role of FGF19 and pro-inflammatory cytokines has on this disease state. Methods Serum FGF19, IL-6 and, TNF-α were measured in 52 IF patients at median age 6.0 years (IQR 2.2–13) after 10 months (4.1–39) on parenteral nutrition (PN). Thirty-nine patients underwent liver biopsies. Results In IF patients, FGF19 concentrations were lower and those of IL-6 and TNF-α higher compared to healthy matched controls ( p ⩽0.001 for all). FGF19 concentrations were further decreased in patients without a remaining ileum [37 pg/ml (IQR 30–68) vs. 74 (35–135) p = 0.028], and correlated with remaining ileum length (r = 0.333, p = 0.018) and markers of cholesterol synthesis (r = −0.552 to −0.643, p