论文部分内容阅读
Hepatic fibrosis contributes to adverse outcome in cystic fibrosis (CF). Early detection of CF liver disease (CFLD) may identify patients at risk of significant complications. To evaluate the utility of serum markers to detect hepatic fibrosis in children with CFLD vs. CF patients without liver disease (CFnoLD) and controls. Sera from 36 CFLD, 30 CFnoLD and 39 controls were assessed for tissue inhibitor of matrix metalloproteinase (MMP) (TIMP)-1, collagen (CL) IV, MMP-2, hyaluronic acid (HA) and prolyl hydroxylase (PH) by enzyme immunoassay and were correlated with hepatic fibrosis score in CFLD. TIMP-1, PH and CL IV were increased in CFLD vs. CFnoLD and controls. Fibrosis score was negatively correlated with TIMP 1 (r=-0.34, P=0.06) and PH(r=-0.48, P=0.008). Receiver operating characteristics analysis showed CL IV (AUC 0.785, P<0.0001) and TIMP-1 (AUC 0.765, P<0.0001) differentiated CFLD from CFnoLD and controls, while PH (AUC 0.814, P < 0.0001) predicted early fibrogenesis. Diagnostic accuracy improved using logistic regression combining (i) CL IV, TIMP-1, PH to identify CFLD (AUC 0.831, P< 0.0001) and (ii) TIMP-1, PH to identify CFLD patients with no fibrosis (AUC 0.852, P < 0.02). Elevated TIMP-1, CL IV, PH may be indicators of hepatic fibrogenesis in CF. Increased TIMP-1, PH may be early markers of CFLD.
Early evaluation of CF liver disease (CFLD) may identify patients at risk of significant complications. To evaluate the utility of serum markers to detect hepatic fibrosis in children with CFLD vs. CF patients Sera from 36 CFLD, 30 CFnoLD and 39 controls were assessed for tissue inhibitor of matrix metalloproteinase (MMP) (TIMP) -1, collagen (CL) IV, MMP-2, hyaluronic acid ) and prolyl hydroxylase (PH) by enzyme immunoassay and were correlated with hepatic fibrosis score in CFLD. TIMP-1, PH and CL IV were increased in CFLD vs. CFnoLD and controls. Fibrosis score was negatively correlated with TIMP 1 (r = Receiver operating characteristics analysis showed CL IV (AUC 0.785, P <0.0001) and TIMP-1 (AUC 0.765, P <0.0001) differentiated CFLD from CF noLD (0.34, P = 0.06) and PH (r = -0.48, and controls, while PH (AUC 0.814, P <0.0001) predicted early fibrogenesis. Diagnosti PHI to identify CFLD patients with no fibrosis (AUC 0.852, P (p <0.0001) and (ii) TIMP-1, PH to identify CFLD patients with no fibrosis <0.02). Elevated TIMP-1, CL IV, PH may be indicators of hepatic fibrogenesis in CF. Increased TIMP-1, PH may be early markers of CFLD.