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Liver Ultrasound Patterns in Children with Cystic Fibrosis Correlate with Non-Invasive Tests of Liver Disease

Ling, Simon C.*; Ye, Wen; Leung, Daniel H.; Navarro, Oscar M.§; Weymann, Alexander||; Karnsakul, Wikrom; Freeman, A. Jay#; Magee, John C.**; Narkewicz, Michael R.††

Journal of Pediatric Gastroenterology and Nutrition: June 7, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPG.0000000000002413
Original Article: Hepatology: PDF Only
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Objectives: Early identification of children with cystic fibrosis (CF) at risk for severe liver disease (CFLD) would enable targeted study of preventative therapies. There is no gold standard test for CFLD. Ultrasonography (US) is used to identify CFLD, but with concerns for its diagnostic accuracy. We aim to determine if differences in standard blood tests, imaging variables and non-invasive liver fibrosis indices correlate with liver US patterns and thus provide supportive evidence that a heterogeneous US liver pattern reflects clinically relevant liver disease.

Methods: We studied baseline research abdominal US and bloodwork from 244 children with pancreatic insufficient CF, ages 3–12y, enrolled in a prospective study of the ability of US to predict CF cirrhosis (PUSH study). Children with a heterogeneous liver pattern on US (HTG, n = 62) were matched 1:2 in design with children with normal US (NL, n = 122). Analyses included children with nodular (NOD, n = 22) and homogeneous hyperechoic (HMG, n = 38) livers.

Results: Univariate analysis showed significant differences between US groups for standard blood tests, spleen size, and non-invasive liver fibrosis indices. Multivariable models discriminated NOD vs. NL with excellent accuracy (AUROC 0.96). Models also distinguish HTG vs. NL (AUROC 0.76), NOD vs. HTG (0.78), and HMG vs NL (0.79).

Conclusion: Liver US patterns in children with CF correlate with platelet count, spleen size and indices of liver fibrosis. Multivariable models of these biomarkers have excellent discriminating ability for NL vs NOD, and good ability to distinguish other US patterns, suggesting that US patterns correlate with clinically relevant liver disease.

*The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada

University of Michigan, Ann Arbor, MI, United States

Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States

§Department of Diagnostic Imaging, The Hospital for Sick Children and Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada

||Washington University School of Medicine, St Louis, MO, United States

Johns Hopkins University School of Medicine, Baltimore, MD, United States

#Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA, United States

**University of Michigan Medical School, Ann Arbor, MI, United States

††Digestive Health Institute, Children's Hospital Colorado & University of Colorado School of Medicine, Aurora, CO, United States.

Address correspondence and reprint requests to Simon C. Ling, MBChB, The Hospital for Sick Children, Room 8418 Black Wing, 555 University Avenue, Toronto, Ontario M5G1X8, Canada (e-mail: simon.ling@sickkids.ca).

Received 16 November, 2018

Accepted 24 April, 2019

for the Cystic Fibrosis Liver Disease Research Network (CFLD-NET)

Grant support: The NIDDK (Grant Number U01 DK062456)

The Cystic Fibrosis Foundation (Grant Number NARKEW07A0)

Disclosures:

SC Ling receives research support from Bristol Myers Squibb and Abbvie.

W Ye, no disclosures

DH Leung receives research support from Bristol Myers Squibb, Gilead, Abbvie, and Roche.

O Navarro, no disclosures

A Weymann, no disclosures

W Karnsakul receives research support from Gilead

AJ Freeman, no disclosures

J Magee, no disclosures

MR Narkewicz,

Author contributions:

All authors were involved in study concept and design, acquisition of data, analysis and interpretation of results, drafting of the manuscript.

WY also performed the statistical analyses.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org).

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,