title: Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis.
abstract: OBJECTIVE
  To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF).
  
  
  METHODS
  Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and US or Toronto CF registries. χ(2) or ANOVA were used to compare variables among ultrasound groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal ultrasound.
  
  
  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 = .0004), homogeneous (P < .0001), and heterogeneous (P = .03) were older than normal. More males were heterogeneous (P = .001). More heterogeneous (15.0%, P = .009) and cirrhosis (25.0%, P = .005) had CF-related diabetes or impaired glucose tolerance vs normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, P = .0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, P < .0001) and CF-related diabetes (OR 2.21, P = .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 ultrasound is associated with CF-related diabetes, and early P aeruginosa is associated with normal ultrasound. Prospective assessment of these risk factors may identify potential interventional targets.
  
  
  BACKGROUND
  ClinicalTrials.gov: NCT01144507.
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine/Texas Children's Hospital, Houston, TX. Electronic address: dhleung@texaschildrens.org.
authors.forename: Daniel H
authors.initials: DH
authors.lastname: Leung
authors.affiliation: Department of Biostatistics, University of Michigan, Ann Arbor, MI.
authors.forename: Wen
authors.initials: W
authors.lastname: Ye
authors.affiliation: Pediatric Gastroenterology, Hepatology and Nutrition, Indiana University School of Medicine Riley Hospital for Children, Indianapolis, IN.
authors.forename: Jean P
authors.initials: JP
authors.lastname: Molleston
authors.affiliation: Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Washington University School of Medicine, St. Louis, MO.
authors.forename: Alexander
authors.initials: A
authors.lastname: Weymann
authors.affiliation: Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, and Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Simon
authors.initials: S
authors.lastname: Ling
authors.affiliation: Division of Pediatric Pulmonology, Johns Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Shruti M
authors.initials: SM
authors.lastname: Paranjape
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine, Atlanta, GA.
authors.forename: Rene
authors.initials: R
authors.lastname: Romero
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Minnesota Masonic Children's Hospital, Minneapolis, MN.
authors.forename: Sara Jane
authors.initials: SJ
authors.lastname: Schwarzenberg
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Joseph
authors.initials: J
authors.lastname: Palermo
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert Lurie Children's Hospital, Chicago, IL.
authors.forename: Estella M
authors.initials: EM
authors.lastname: Alonso
authors.affiliation: Division of Gastroenterology and Hepatology, Seattle Children's Hospital and University of Washington, Seattle, WA.
authors.forename: Karen F
authors.initials: KF
authors.lastname: Murray
authors.affiliation: Cystic Fibrosis Foundation, Bethesda, MD.
authors.forename: Bruce C
authors.initials: BC
authors.lastname: Marshall
authors.affiliation: Liver Diseases Research Branch, Division of Digestive Diseases and Nutrition, NIDDK, Bethesda, MD.
authors.forename: Averell H
authors.initials: AH
authors.lastname: Sherker
authors.affiliation: Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO.
authors.forename: Marilyn J
authors.initials: MJ
authors.lastname: Siegel
authors.affiliation: EB Singleton Department of Diagnostic Imaging, Texas Children's Hospital, Houston, TX.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Krishnamurthy
authors.affiliation: Department of Radiology, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO.
authors.forename: Roger
authors.initials: R
authors.lastname: Harned
authors.affiliation: Pediatric Radiology, Riley Hospital for Children, Indianapolis, IN.
authors.forename: Boaz
authors.initials: B
authors.lastname: Karmazyn
authors.affiliation: Department of Surgery, University of Michigan, Ann Arbor, MI.
authors.forename: John C
authors.initials: JC
authors.lastname: Magee
authors.affiliation: Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado SOM and Children's Hospital Colorado, Aurora, CO. Electronic address: michael.narkewicz@childrenscolorado.org.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Narkewicz
mesh_terms: D000704:Analysis of Variance; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications / Q000000981:diagnostic imaging*; D008297:Male; D009752:Nutritional Status; D011446:Prospective Studies; D011552:Pseudomonas Infections / Q000150:complications; D011550:Pseudomonas aeruginosa; D012307:Risk Factors; D014463:Ultrasonography; D014580:Ursodeoxycholic Acid / Q000737:chemistry
chemical_list: D014580:Ursodeoxycholic Acid