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J Cyst Fibros. 2019 Jul 11. pii: S1569-1993(19)30820-3. doi: 10.1016/j.jcf.2019.06.017. [Epub ahead of print]

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

Author information

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.

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

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

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