Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, 300 Herston Rd, Herston, QLD 4006, Australia; Faculty of Medicine, The University of Queensland, Brisbane, QLD 4006, Australia.
Department of Gastroenterology and Hepatology, Queensland Children's Hospital, 501 Stanley St, South Brisbane, QLD, 4101, Australia.
J Cyst Fibros. 2020 May;19(3):449-454. doi: 10.1016/j.jcf.2019.06.017. Epub 2019 Jul 11.
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.
目前,囊性纤维化(CF)相关肝病(CFLD)的诊断方法是非特异性的,并且在出现门静脉高压等晚期疾病之前,很难评估疾病的进展情况。本研究旨在探讨超声剪切波弹性成像(SSWE)在诊断 CF 儿童 CFLD 及评估肝纤维化严重程度方面的潜在应用。
本研究共纳入 125 名儿童,包括 CFLD 组(n=55)、无肝疾病证据的 CF 患者(CFnoLD,n=41)和对照组(n=29)。采用临床、生化和影像学最佳实践指南诊断 CFLD。采用超声检查诊断门静脉高压和/或大结节性肝硬化确立晚期 CFLD。采用 SSWE 检测肝脏硬度值(LSM),评估 LSM 单独或与天门冬氨酸氨基转移酶与血小板比值指数(APRI)联合诊断 CFLD 的性能。
CFLD 组 LSM 显著高于 CFnoLD 组(8.1kPa,IQR=6.7-11.9)和对照组(5.3kPa,IQR=4.9-5.8;P<0.0001),CFnoLD 组也高于对照组(P=0.0192)。ROC 曲线分析显示,LSM 以 6.85kPa 为截断值诊断 CFLD 的诊断准确性较高,AUC=0.79(敏感度=75%,特异度=71%,P<0.0001)。APRI 也能区分 CFLD(AUC=0.74,P=0.004)。联合 LSM+APRI 的分类回归树模型显示,准确预测 CFLD 的可能性增加了 14.8 倍(AUC=0.84)。SSWE 以 9.05kPa 为截断值诊断晚期疾病的诊断准确性极佳(AUC=0.95,P<0.0001)。
SSWE 测定的 LSM 在诊断 CF 儿童 CFLD 方面具有较好的诊断准确性,与 APRI 联合应用可提高诊断准确性。SSWE 可单独区分晚期 CFLD。