Justus-Liebig-University Giessen, Department of Internal Medicine, Division of Gastroenterology, 35385 Giessen, Germany.
Dig Liver Dis. 2012 Sep;44(9):780-7. doi: 10.1016/j.dld.2012.04.008. Epub 2012 May 29.
Cystic fibrosis (CF) associated liver disease develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools are lacking.
We aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CF patients.
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.
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.
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.
囊性纤维化（CF）相关肝病在约 30%的 CF 患者中发展。然而，缺乏常规的敏感诊断工具。
我们旨在比较瞬时弹性成像和实验性纤维化标志物在 CF 患者肝病检测中的价值。
对 145 例 CF 患者（75 例儿童，70 例成人）进行前瞻性研究，并接受瞬时弹性成像检查。根据最近的指南诊断 CF 肝病。通过 ELISA 测定 YKL-40、HA、PIIIP、MMP-9、TIMP-1 和 TIMP-2 的血清浓度。
与无 CF 肝病的患者相比，有 CF 肝病的成人和儿童的瞬时弹性成像增加，对 CF 肝病具有较高的诊断准确性。在有门脉高压的成年 CF 患者中，弹性成像进一步增强。TIMP-2 在伴有 CF 肝病相关门脉高压的成年 CF 患者中升高，对成年 CF 肝病和儿童 CF 肝病具有较高的诊断准确性。TIMP-1 在成年 CF 肝病患者中有较高的诊断准确性。当将弹性成像和各自的生物标志物结合起来检测 CF 肝病和门脉高压时，诊断敏感性增加。
TIMP-1 和 TIMP-2 是 CF 相关肝病和门脉高压的有力生物标志物。它们的测定可以确认并提高瞬时弹性成像的诊断准确性。
