Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

• Type of Material: Article
• Authors: Daniel H. Leung, Baylor College of Medicine; Wen Ye, University of Michigan; Jean P. Molleston, Indiana University; Alexander Weymann, Washington University; Simon Ling, University of Toronto; Shruti M. Paranjape, Johns Hopkins University; Rene Romero, Emory University; Sara Jane Schwarzenberg, University of Minnesota; Joseph Palermo, Cincinnati Children's Hospital Medical Center; Estella M. Alonso, Ann & Robert Lurie Children's Hospital; Karen F. Murray, University of Washington; Bruce C. Marshall, Cystic Fibrosis Foundation; Averell H. Sherker, National Institute of Diabetes and Digestive and Kidney Diseases; Marilyn J. Siegel, Washington University; Rajesh Krishnamurthy, Texas Children's Hospital; Roger Harned, University of Colorado; Boaz Karmazyn, Riley Hospital for Children; John C. Magee, University of Michigan; Michael R. Narkewicz, University of Colorado
• Language: English
• Date: 2015-10-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2015 Elsevier Inc.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.jpeds.2015.06.062
• Title of Journal or Parent Work: Journal of Pediatrics
• ISSN: 0022-3476
• Volume: 167
• Issue: 4
• Start Page: 862
• End Page: +
• Grant/Funding Information: Supported by the Cystic Fibrosis Foundation (NARKEW07A0 [Colorado]; DK062453 and DK62456 [The University of Michigan]), the National Institutes of Health (NIH)/National Center for Advancing Translational Sciences (NCATS; UL1 TR001082 [Colorado]; UL1 TR000077 [Cincinnati], DK084536-07; UL1 TR000150 [Northwestern University]), Clinical & Translational Science Institute (CTSI UL1TR001108 [Indiana University]), Institute for Clinical & Translational Research/NCATS/NIH (UL1 TR 001079 [Johns Hopkins]), NIH/NUCATS/Institute of Translational Health Sciences (RR025014), and NIH (Clinical and Translational Science Award TR000423 [University of Washington]).
• Keywords: hepatobiliary; risk; FATTY LIVER-DISEASE; imaging; CFTR dysfunction; Pediatrics; Life Sciences & Biomedicine; sonography; Science & Technology
• Research Categories: Health Sciences, Radiology; Health Sciences, Medicine and Surgery

Abstract

Objective: To investigate the relationship between abdominal ultrasound (US) findings and demographic, historical and clinical features in children with CF. Study design: Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multi-center study of US to predict hepatic fibrosis. Consensus US patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and U.S. or Toronto CF registries. Chi-square or ANOVA were used to compare variables among US groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal US. Results: Findings in 719 subjects were normal (n=590, 82.1%), heterogeneous (64, 8.9%), homogeneous (41, 5.7%), and cirrhosis (24, 3.3%). Cirrhosis (p=0.0004), homogeneous (p<0.0001) and heterogeneous (p=0.03) were older than normal. More males were heterogeneous (p=0.001). More heterogeneous (15.0%, p=0.009) and cirrhosis (25.0%, p=0.005) had CF-related diabetes or impaired glucose tolerance versus normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, p=0.0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, p <0.0001) and CF-related diabetes (OR 2.21, p=0.019) were associated with increased risk of abnormal. Conclusions: Unsuspected cirrhosis is seen in 3.3% of young patients with CF, heterogeneous in 8.9%. abnormal US is associated with CF-related diabetes, and early P aeruginosa is associated with normal US. Prospective assessment of these risk factors may identify potential interventional targets.