Abstract
Background
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.
Objective
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.
Materials and methods
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.
Results
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.
Conclusion
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.
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Acknowledgments
Funding was provided by the Cystic Fibrosis Foundation (SELLER16L0, SELLER19GE0 to Z.M.S), National Institute of Diabetes and Digestive and Kidney Diseases (K08DK124684 to Z.M.S.) and Stanford University (Z.M.S). Dr. Shreyas Vasanawala (Stanford University, Palo Alto, CA) assisted in generating the MR elastography confidence overlay figure.
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Supplementary Information
Online Supplementary Material 1
Scatter plots show the Pearson correlation coefficient of shear-wave elastography (SWE) and magnetic resonance (MR) elastography using the linear 9 L4 probe in point SWE (a) and in 2-D SWE (b) (EPS 182572 kb).
Online Supplementary Material 2
Scatter plots show the median (line) comparing US qualitative patterns with shear-wave elastography (SWE). Significance was determined by comparing nodular to normal, and nodular to echogenic using the Student’s t test. The dotted line represents optimal cut-off as determined by the Youden index (PNG 98 kb).
Online Supplementary Material 3
Scatter plots show the Pearson correlation coefficient of shear-wave elastography (SWE) and magnetic resonance (MR) elastography using the curved 6C1 probe at 6 MHz separated based on body mass index (BMI) percentile (a and b) and age (c and d) (EPS 200324 kb).
Online Supplementary Material 4
Plots depict each patient’s median (dot) and minimum/maximum (whiskers) reported 9-MHz point shear-wave elastography (SWE) (a) and 9-MHz 2-D SWE (b) values, separated by MR elastography score (vertical dotted lines). c Confidence overlay for the image shown in Fig. 3 (EPS 180817 kb).
Online Supplementary Material 6
(DOXC 13.6 kb).
Online Supplementary Material 6
Plots show the Pearson correlation coefficient of shear-wave elastography (SWE) and aspartate aminotransferase (AST)-to-platelet ratio index (APRI) (a), fibrosis-4 (FIB-4) (b), and gamma-glutamyl transferase (GGT)-to-platelet ratio (GPR) (c) (TIF 5303 kb)
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Levitte, S., Lee, L.W., Isaacson, J. et al. Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis. Pediatr Radiol 51, 1369–1377 (2021). https://doi.org/10.1007/s00247-021-05015-w
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DOI: https://doi.org/10.1007/s00247-021-05015-w