Noninvasive measurement of liver stiffness with transient elastography has been recently validated for the evaluation of hepatic fibrosis in chronic hepatitis C. The current study assessed the diagnostic performance o...Noninvasive measurement of liver stiffness with transient elastography has been recently validated for the evaluation of hepatic fibrosis in chronic hepatitis C. The current study assessed the diagnostic performance of liver stiffness measurement(LSM) for the determination of fibrosis stage in chronic cholestatic diseases. One hundred one patients with primary biliary cirrhosis(PBC, n = 73) or primary sclerosing cholangitis(PSC, n = 28) were prospectively enrolled in a multicenter study. All patients underwent liver biopsy(LB) and LSM. Histological and fibrosis stages were assessed on LB by two pathologists. LSM was performed by transient elastography. Efficiency of LSM for the determination of histological and fibrosis stages were determined by a receiver operating characteristics(ROC) curve analysis. Analysis failed in six patients(5.9%) because of unsuitable LB (n = 4) or LSM(n = 2). Stiffness values ranged from 2.8 to 69.1 kPa (median, 7.8 kPa). LSM was correlated to both fibrosis(Spearman’s p = 0.84, P < .0001) and histological(0.79, P < .0001) stages. These correlations were still found when PBC and PSC patients were analyzed separately. Areas under ROC curves were 0.92 for fibrosis stage (F) ≥2,0.95 for F ≥3 and 0.96 for F = 4. Optimal stiffness cutoff values of 7.3, 9.8, and 17.3 kPa showed F ≥2, F ≥3 and F = 4, respectively. LSM and serum hyaluronic acid level were independent parameters associated with extensive fibrosis on LB. In conclusion, transient elastography is a simple and reliable noninvasive means for assessing biliary fibrosis. It should be a promising tool to assess antifibrotic therapies in PBC or PSC.展开更多
Objectives: To evaluate the risk for atherosclerosis in Alagille syndrome (AG S) and progressive familial intrahepatic cholestasis (PFIC) on the basis of lipo protein metabolism and by ultrasonography. Study design: F...Objectives: To evaluate the risk for atherosclerosis in Alagille syndrome (AG S) and progressive familial intrahepatic cholestasis (PFIC) on the basis of lipo protein metabolism and by ultrasonography. Study design: Five patients with AGS and 5 with PFIC, ages 3 to 4 years, were enrolled. Intimal- medial thickness an d wall stiffness of the common carotid artery were examined by ultrasonography. Serum levels of lipids and lipoproteins were determined. Further, the chemical c omposition of LDL and its ability to transform macrophages into foam cells were determined. Results: Intimal- medial thickness and wall stiffness were increase d in patients with PFIC but not in patients with AGS. Total cholesterol,LDL chol esterol,HDL cholesterol, and lipoprotein X were remarkably increased in patients with AGS, whereas in patients with PFIC, an increase in triglyceride and a decr ease in HDL cholesterolwere the prominent findings. However, despite the normal LDL cholesterol level, oxidized LDL level was strikingly high in patients with P FIC. LDLs from patients with PFIC had high TG contents and exhibited high abilit ies to transform macrophages into foam cells. Conclusions: These findings sugges t that patients with PFIC are at high risk for cardiovascular disorders involvin g atherosclerosis.展开更多
文摘Noninvasive measurement of liver stiffness with transient elastography has been recently validated for the evaluation of hepatic fibrosis in chronic hepatitis C. The current study assessed the diagnostic performance of liver stiffness measurement(LSM) for the determination of fibrosis stage in chronic cholestatic diseases. One hundred one patients with primary biliary cirrhosis(PBC, n = 73) or primary sclerosing cholangitis(PSC, n = 28) were prospectively enrolled in a multicenter study. All patients underwent liver biopsy(LB) and LSM. Histological and fibrosis stages were assessed on LB by two pathologists. LSM was performed by transient elastography. Efficiency of LSM for the determination of histological and fibrosis stages were determined by a receiver operating characteristics(ROC) curve analysis. Analysis failed in six patients(5.9%) because of unsuitable LB (n = 4) or LSM(n = 2). Stiffness values ranged from 2.8 to 69.1 kPa (median, 7.8 kPa). LSM was correlated to both fibrosis(Spearman’s p = 0.84, P < .0001) and histological(0.79, P < .0001) stages. These correlations were still found when PBC and PSC patients were analyzed separately. Areas under ROC curves were 0.92 for fibrosis stage (F) ≥2,0.95 for F ≥3 and 0.96 for F = 4. Optimal stiffness cutoff values of 7.3, 9.8, and 17.3 kPa showed F ≥2, F ≥3 and F = 4, respectively. LSM and serum hyaluronic acid level were independent parameters associated with extensive fibrosis on LB. In conclusion, transient elastography is a simple and reliable noninvasive means for assessing biliary fibrosis. It should be a promising tool to assess antifibrotic therapies in PBC or PSC.
文摘Objectives: To evaluate the risk for atherosclerosis in Alagille syndrome (AG S) and progressive familial intrahepatic cholestasis (PFIC) on the basis of lipo protein metabolism and by ultrasonography. Study design: Five patients with AGS and 5 with PFIC, ages 3 to 4 years, were enrolled. Intimal- medial thickness an d wall stiffness of the common carotid artery were examined by ultrasonography. Serum levels of lipids and lipoproteins were determined. Further, the chemical c omposition of LDL and its ability to transform macrophages into foam cells were determined. Results: Intimal- medial thickness and wall stiffness were increase d in patients with PFIC but not in patients with AGS. Total cholesterol,LDL chol esterol,HDL cholesterol, and lipoprotein X were remarkably increased in patients with AGS, whereas in patients with PFIC, an increase in triglyceride and a decr ease in HDL cholesterolwere the prominent findings. However, despite the normal LDL cholesterol level, oxidized LDL level was strikingly high in patients with P FIC. LDLs from patients with PFIC had high TG contents and exhibited high abilit ies to transform macrophages into foam cells. Conclusions: These findings sugges t that patients with PFIC are at high risk for cardiovascular disorders involvin g atherosclerosis.
