Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease.

Liver disease frequently complicates cystic fibrosis (CF), with CF liver disease (CFLD) a leading cause of death. Liver biopsy is rarely performed because of the patchy nature of the disease. Transient elastography can reliably stage liver fibrosis via liver stiffness measurement (LSM). To evaluate LSM as a diagnostic tool in adults with CFLD. Fifty adult patients with CF were prospectively studied: 25 with CFLD and 25 without CFLD. The presence of CFLD and portal hypertension (PHT) was assessed according to strict established criteria based on serial biochemistry and imaging. All patients underwent LSM; APRI, Hepascore(®) and Forns score were calculated. Median LSM was higher in those with CFLD [8.1 kPa (IQR 6.8-9.5) vs. 5.0 kPa (IQR 4.1-5.6); P < 0.001]. On multivariate analysis, LSM was the only variable associated with CFLD (OR 2.74, 95% CI 1.53-4.89; P = 0.001). AUROC for LSM predicting CFLD was 0.87 (95% CI 0.77-0.98) and an LSM ≥ 6.8 kPa predicted CFLD with 76.0% sensitivity and 92.0% specificity. Median LSM was higher in those with PHT [15.7 kPa (IQR 9.2-17.2) vs. 5.4 kPa (IQR 4.3-6.8); P < 0.001]. The AUROC for LSM predicting the presence of PHT was 0.96 (95% CI 0.92-1.00). An LSM cut-off of ≥ 8.9 kPa predicted the presence of PHT with 87.5% sensitivity, 90.5% specificity, 63.6% positive predictive value and 92.9% negative predictive value. LSM is an accurate and reliable non-invasive tool in assessing CFLD and PHT. An LSM ≥ 6.8 kPa is highly suggestive of CFLD and an LSM <8.9 kPa reliably excludes PHT.

Kitson MT ，Kemp WW ，Iser DM ，Paul E ，Wilson JW ，Roberts SK ... -  《-》

Noninvasive methods, including transient elastography, for the detection of liver disease in adults with cystic fibrosis.

Sadler MD ，Crotty P ，Fatovich L ，Wilson S ，Rabin HR ，Myers RP ... -  《-》

Correlation of Transient Elastography With Severity of Cystic Fibrosis-related Liver Disease.

Aqul A ，Jonas MM ，Harney S ，Raza R ，Sawicki GS ，Mitchell PD ，Fawaz R ... -  《-》

Baseline and serial liver stiffness measurement in prediction of portal hypertension progression for patients with compensated cirrhosis.

Liver stiffness measurement (LSM) using transient elastography is useful in prediction of significant portal hypertension (PHT). To evaluate the usefulness of baseline and serial LSM in predicting clinical disease progression (CDP) for patients with compensated hepatic cirrhosis. Consecutive patients with compensated cirrhosis and without hepatocellular carcinoma (HCC) were prospectively enrolled. Baseline LSM was assessed at enrolment, then at a 6- to 12-month interval. Esophagogastroduodenoscopy and ultrasonography were performed regularly for surveillance of varices and HCC, while CDP including HCC development and PHT progression was recorded. Two hundred and twenty patients were enrolled. In a median follow-up of 36.9 months, CDP were detected in 49 patients including 30 PHT progression and 19 HCC developments. The cumulative incidence of CDP, PHT progression and HCC development at 3 years was 20.7%, 12.8% and 9.1% respectively. Multivariate analyses showed that baseline LSM was an independent predictor of PHT progression and CDP, however, not of HCC occurrence. The performance of baseline LSM in predicting PHT progression, varices growth/development and hepatic decompensation was 0.744, 0.638 and 0.929. With 17, 12 and 21.1 kPa as the cut-off, the negative predictive value was 92, 94 and 99% respectively. Patients with baseline LSM ≧17 kPa without serial changes had higher risk of PHT progression. For patients with compensated cirrhosis, LSM was an independent predictor of PHT progression and CDP, but not of HCC occurrence. Baseline LSM was useful to exclude PHT progression. Patients with baseline and serial LSM ≧17 kPa had higher risk of PHT progression.

Wang JH ，Chuah SK ，Lu SN ，Hung CH ，Kuo CM ，Tai WC ，Chiou SS ... -  《-》

Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis.

Current diagnostic methods for the diagnosis of Cystic fibrosis (CF)-associated liver disease (CFLD) are non-specific and assessment of disease progression is difficult prior to the advent of advanced disease with portal hypertension. This study investigated the potential of Supersonic shear-wave elastography (SSWE) to non-invasively detect CFLD and assess hepatic fibrosis severity in children with CF. 125 children were enrolled in this study including CFLD (n = 55), CF patients with no evidence of liver disease (CFnoLD = 41) and controls (n = 29). CFLD was diagnosed using clinical, biochemical and imaging best-practice guidelines. Advanced CFLD was established by the presence of portal hypertension and/or macronodular cirrhosis on ultrasound. Liver stiffness measurements (LSM) were acquired using SSWE and diagnostic performance for CFLD detection was evaluated alone or combined with aspartate aminotransferase-to-platelet ratio index (APRI). LSM was significantly higher in CFLD (8.1 kPa, IQR = 6.7-11.9) versus CFnoLD (6.2 kPa, IQR = 5.6-7.0; P < 0.0001) and Controls (5.3 kPa, IQR = 4.9-5.8; P < 0.0001). LSM was also increased in CFnoLD versus Controls (P = 0.0192). Receiver Operating Characteristic (ROC) curve analysis demonstrated good diagnostic accuracy for LSM in detecting CFLD using a cut-off = 6.85 kPa with an AUC = 0.79 (Sensitivity = 75%, Specificity = 71%, P < 0.0001). APRI also discriminated CFLD (AUC = 0.74, P = 0.004). Classification and regression tree modelling combining LSM + APRI showed 14.8 times greater odds of accurately predicting CFLD (AUC = 0.84). The diagnostic accuracy of SSWE for discriminating advanced disease was excellent with a cut-off = 9.05 kPa (AUC = 0.95; P < 0.0001). SSWE-determined LSM shows good diagnostic accuracy in detecting CFLD in children, which was improved when combined with APRI. SSWE alone discriminates advanced CFLD.

Calvopina DA ，Noble C ，Weis A ，Hartel GF ，Ramm LE ，Balouch F ，Fernandez-Rojo MA ，Coleman MA ，Lewindon PJ ，Ramm GA ... -  《-》