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

Diego A Calvopina; Charlton Noble; Anna Weis; Gunter F Hartel; Louise E Ramm; Fariha Balouch; Manuel A Fernandez-Rojo; Miranda A Coleman; Peter J Lewindon; Grant A Ramm

doi:10.1016/j.jcf.2019.06.017

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

J Cyst Fibros. 2020 May;19(3):449-454. doi: 10.1016/j.jcf.2019.06.017. Epub 2019 Jul 11.

Authors

Affiliations

¹ 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. Electronic address: Diego.Calvopina@qimrberghofer.edu.au.
² Department of Gastroenterology and Hepatology, Queensland Children's Hospital, 501 Stanley St, South Brisbane, QLD, 4101, Australia. Electronic address: Charlton.Noble@health.qld.gov.au.
³ 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. Electronic address: Anna.Weis@qimrberghofer.edu.au.
⁴ QIMR Berghofer Statistics Unit, QIMR Berghofer Medical Research Institute, 300 Herston Rd, Herston, QLD, 4006, Australia. Electronic address: Gunter.Hartel@qimrberghofer.edu.au.
⁵ Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, 300 Herston Rd, Herston, QLD 4006, Australia. Electronic address: Louise.Ramm@qimrberghofer.edu.au.
⁶ Department of Gastroenterology and Hepatology, Queensland Children's Hospital, 501 Stanley St, South Brisbane, QLD, 4101, Australia. Electronic address: Fariha.Balouch@health.qld.gov.au.
⁷ Faculty of Medicine, The University of Queensland, Brisbane, QLD 4006, Australia; Madrid Institute for Advanced Studies (IMDEA) in Food, CEI UAM+CSIC, Hepatic , Ctra Canto Blanco, 8, Regenerative Medicine Group, Madrid 28049, Spain. Electronic address: manuel.fernandez@imdea.org.
⁸ Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, 300 Herston Rd, Herston, QLD 4006, Australia.
⁹ 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. Electronic address: Peter.Lewindon@health.qld.gov.au.
¹⁰ 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. Electronic address: Grant.Ramm@qimrberghofer.edu.au.

PMID: 31303380
DOI: 10.1016/j.jcf.2019.06.017

Abstract

Background: 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.

Methods: 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).

Results: 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).

Conclusions: SSWE-determined LSM shows good diagnostic accuracy in detecting CFLD in children, which was improved when combined with APRI. SSWE alone discriminates advanced CFLD.

Keywords: Cirrhosis; Hepatic fibrosis; Non-invasive assessment of liver disease; children's cholestatic liver disease.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Aspartate Aminotransferases / blood*
Biomarkers / blood
Child
Cystic Fibrosis* / blood
Cystic Fibrosis* / complications
Cystic Fibrosis* / physiopathology
Disease Progression
Elasticity Imaging Techniques / methods*
Female
Humans
Liver Cirrhosis* / blood
Liver Cirrhosis* / diagnosis
Liver Cirrhosis* / etiology
Liver* / diagnostic imaging
Liver* / physiopathology
Male
Patient Acuity
Platelet Count / methods*
Prognosis
Reproducibility of Results
Severity of Illness Index

Substances

Biomarkers
Aspartate Aminotransferases