Department of Pediatrics, Stanford University, Palo Alto, CA, USA.
Department of Pediatric Radiology, Stanford University, Palo Alto, CA, USA.
Pediatr Radiol. 2021 Jul;51(8):1369-1377. doi: 10.1007/s00247-021-05015-w. Epub 2021 Mar 24.
Complications from liver cirrhosis are a leading cause of death in children with cystic fibrosis. Identifying children at risk for developing liver cirrhosis and halting its progression are critical to reducing liver-associated mortality.
Quantitative US imaging, such as shear-wave elastography (SWE), might improve the detection of liver fibrosis in children with cystic fibrosis (CF) over gray-scale US alone. We incorporated SWE in our pediatric CF liver disease screening program and evaluated its performance using magnetic resonance (MR) elastography.
Ninety-four children and adolescents with CF underwent 178 SWE exams, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT) and platelet measurements. Of these, 27 children underwent 34 MR elastography exams. We evaluated SWE performance using 6-MHz and 9-MHZ point SWE, and 9-MHz two-dimensional (2-D) SWE.
The 6-MHz point SWE was the only method that correlated with MR elastography (r=0.52; 95% confidence interval [CI] 0.20-0.74; P=0.003). SWE of 1.45 m/s distinguished normal from abnormal MR elastography (79% sensitivity, 100% specificity, 100% positive predictive value [PPV], 55% negative predictive value [NPV], area under the receiver operating characteristic [AUROC] curve 0.94). SWE of 1.84 m/s separated mild-moderate (3.00-4.77 kPa) from severe (>4.77 kPa) MR elastography (88% sensitivity, 86% specificity, 78% PPV, 93% NPV, AUROC 0.79). Elevations of AST, ALT, GGT and thrombocytopenia were associated with higher SWE. AST-to-platelet ratio index of 0.42, fibrosis-4 of 0.29, and GGT-to-platelet ratio of 1.43 all had >95% NPV for SWE >1.84 m/s.
Given its correlation with MR elastography, SWE might be a clinically useful predictor of liver fibrosis. We identified imaging criteria delineating the use of SWE to identify increased liver stiffness in children with CF. With multicenter validation, these data might be used to improve the detection and monitoring of liver fibrosis in children with CF.
肝硬化并发症是导致囊性纤维化(CF)儿童死亡的主要原因。识别有发生肝硬化风险的儿童并阻止其进展对于降低与肝脏相关的死亡率至关重要。
定量超声成像(如剪切波弹性成像[SWE])可能优于单独使用灰阶超声来提高对 CF 儿童肝纤维化的检测。我们将 SWE 纳入我们的儿科 CF 肝病筛查计划,并使用磁共振弹性成像(MRE)评估其性能。
94 名儿童和青少年 CF 患者接受了 178 次 SWE 检查、天门冬氨酸氨基转移酶(AST)、丙氨酸氨基转移酶(ALT)、γ-谷氨酰转移酶(GGT)和血小板测量。其中 27 名儿童接受了 34 次 MRE 检查。我们使用 6MHz 和 9MHz 点 SWE 以及 9MHz 二维(2-D)SWE 评估 SWE 性能。
6MHz 点 SWE 是唯一与 MRE 相关的方法(r=0.52;95%置信区间[CI]0.20-0.74;P=0.003)。SWE 为 1.45m/s 可区分正常和异常 MRE(79%的灵敏度,100%的特异性,100%的阳性预测值[PPV],55%的阴性预测值[NPV],受试者工作特征曲线下面积[AUROC]为 0.94)。SWE 为 1.84m/s 可区分轻度至中度(3.00-4.77kPa)和重度(>4.77kPa)MRE(88%的灵敏度,86%的特异性,78%的 PPV,93%的 NPV,AUROC 为 0.79)。AST、ALT、GGT 和血小板减少升高与较高的 SWE 相关。AST 与血小板比值指数 0.42、纤维化 4 指数 0.29 和 GGT 与血小板比值 1.43 对 SWE >1.84m/s 的阴性预测值均>95%。
鉴于其与 MRE 的相关性,SWE 可能是预测肝纤维化的一种有用的临床指标。我们确定了用于识别 CF 儿童肝脏硬度增加的成像标准。经过多中心验证,这些数据可用于提高 CF 儿童肝纤维化的检测和监测。