BACKGROUND: Cystic fibrosis (CF) associated liver disease develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools are lacking. AIMS: We aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CF patients. METHODS: 145 CF patients (75 children, 70 adults) were prospectively studied and received transient elastography. CF liver disease was diagnosed according to recent guidelines. Serum concentrations of YKL-40, HA, PIIIP, MMP-9, TIMP-1 and TIMP-2 were determined by ELISA. RESULTS: Transient elastography was increased in adults and children with CF hepatopathy compared to those without and exhibited a high diagnostic accuracy for CF liver disease. In adults with portal hypertension, elastography was further enhanced. TIMP-2 was elevated in adults with CF hepatopathy associated portal hypertension and exhibited a high diagnostic accuracy for portal hypertension in adults and for CF hepatopathy in children. TIMP-1 had a high diagnostic accuracy for CF hepatopathy in adults. Diagnostic sensitivities were increased when elastography and respective biomarkers were combined for the detection of CF hepatopathy and portal hypertension. CONCLUSIONS: TIMP-1 and TIMP-2 represent powerful biomarkers for CF associated liver disease and portal hypertension. Their determination may confirm and improve the diagnostic accuracy of transient elastography.
BACKGROUND:Cystic fibrosis (CF) associated liver disease develops in approximately 30% of CFpatients. However, routine sensitive diagnostic tools are lacking. AIMS: We aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CFpatients. METHODS: 145 CFpatients (75 children, 70 adults) were prospectively studied and received transient elastography. CF liver disease was diagnosed according to recent guidelines. Serum concentrations of YKL-40, HA, PIIIP, MMP-9, TIMP-1 and TIMP-2 were determined by ELISA. RESULTS: Transient elastography was increased in adults and children with CF hepatopathy compared to those without and exhibited a high diagnostic accuracy for CF liver disease. In adults with portal hypertension, elastography was further enhanced. TIMP-2 was elevated in adults with CF hepatopathy associated portal hypertension and exhibited a high diagnostic accuracy for portal hypertension in adults and for CF hepatopathy in children. TIMP-1 had a high diagnostic accuracy for CF hepatopathy in adults. Diagnostic sensitivities were increased when elastography and respective biomarkers were combined for the detection of CF hepatopathy and portal hypertension. CONCLUSIONS:TIMP-1 and TIMP-2 represent powerful biomarkers for CF associated liver disease and portal hypertension. Their determination may confirm and improve the diagnostic accuracy of transient elastography.
Authors: Matthew D Sadler; Pam Crotty; Linda Fatovich; Stephanie Wilson; Harvey R Rabin; Robert P Myers Journal: Can J Gastroenterol Hepatol Date: 2015-04
Authors: Raphael Enaud; Eric Frison; Sophie Missonnier; Aude Fischer; Victor de Ledinghen; Paul Perez; Stéphanie Bui; Michael Fayon; Jean-François Chateil; Thierry Lamireau Journal: Pediatr Res Date: 2021-03-17 Impact factor: 3.953
Authors: Simon Lam; Alberto Nettel-Aguirre; Stephanie Van Biervliet; Elke Roeb; Matthew D Sadler; Mireen Friedrich-Rust; Thomas Karlas; Matthew T Kitson; Jennifer C C deBruyn Journal: J Can Assoc Gastroenterol Date: 2018-07-09
Authors: Timo Rath; Lisa Hage; Marion Kügler; Katrin Menendez Menendez; Reinhart Zachoval; Lutz Naehrlich; Richard Schulz; Martin Roderfeld; Elke Roeb Journal: PLoS One Date: 2013-03-14 Impact factor: 3.240
Authors: Victoria Klotter; Caroline Gunchick; Enno Siemers; Timo Rath; Helge Hudel; Lutz Naehrlich; Martin Roderfeld; Elke Roeb Journal: PLoS One Date: 2017-06-02 Impact factor: 3.240