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J Pediatr. 2015 Oct;167(4):862-868.e2. doi: 10.1016/j.jpeds.2015.06.062. Epub 2015 Aug 5.

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis.

Author information

1
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine/Texas Children's Hospital, Houston, TX. Electronic address: dhleung@texaschildrens.org.
2
Department of Biostatistics, University of Michigan, Ann Arbor, MI.
3
Pediatric Gastroenterology, Hepatology and Nutrition, Indiana University School of Medicine Riley Hospital for Children, Indianapolis, IN.
4
Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Washington University School of Medicine, St. Louis, MO.
5
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, and Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
6
Division of Pediatric Pulmonology, Johns Hopkins University School of Medicine, Baltimore, MD.
7
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine, Atlanta, GA.
8
Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Minnesota Masonic Children's Hospital, Minneapolis, MN.
9
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
10
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert Lurie Children's Hospital, Chicago, IL.
11
Division of Gastroenterology and Hepatology, Seattle Children's Hospital and University of Washington, Seattle, WA.
12
Cystic Fibrosis Foundation, Bethesda, MD.
13
Liver Diseases Research Branch, Division of Digestive Diseases and Nutrition, NIDDK, Bethesda, MD.
14
Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO.
15
EB Singleton Department of Diagnostic Imaging, Texas Children's Hospital, Houston, TX.
16
Department of Radiology, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO.
17
Pediatric Radiology, Riley Hospital for Children, Indianapolis, IN.
18
Department of Surgery, University of Michigan, Ann Arbor, MI.
19
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.

Abstract

OBJECTIVE:

To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF).

STUDY DESIGN:

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.

TRIAL REGISTRATION:

ClinicalTrials.gov: NCT01144507.

PMID:
26254836
PMCID:
PMC4586395
DOI:
10.1016/j.jpeds.2015.06.062
[Indexed for MEDLINE]
Free PMC Article

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