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Clinical Outcomes at 9-10 Years of Age in Children Born with Cystic Fibrosis Transmembrane Conductance Regulator Related Metabolic Syndrome
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  • Clement Ren,
  • Brian J. Carroll,
  • Joshua S. Ostrenga,
  • Aliza K. Fink,
  • Nicholas Antos,
  • Elizabeth A. Cromwell
Clement Ren
The Children's Hospital of Philadelphia

Corresponding Author:[email protected]

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Brian J. Carroll
Medical College of Wisconsin
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Joshua S. Ostrenga
Cystic Fibrosis Foundation
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Aliza K. Fink
Cystic Fibrosis Foundation
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Nicholas Antos
Medical College of Wisconsin
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Elizabeth A. Cromwell
Cystic Fibrosis Foundation
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Abstract

Background and Objectives: There are limited data on cystic fibrosis (CF) transmembrane conductance regulator-related metabolic syndrome (CRMS) outcomes beyond infancy. The goal of this study was to analyze outcomes of infants with CRMS up to the age of 9-10 years using the CF Foundation Patient Registry (CFFPR). Methods: We analyzed data from the CFFPR for individuals with CF and CRMS born between 2010-2020. We classified all patients based on the clinical diagnosis reported by the CF care center and the diagnosis using CFF guideline definitions for CF and CRMS, classifying children into groups based on agreement between clinical report and guideline criteria. Descriptive statistics for the cohort were calculated for demographics, nutritional outcomes, and microbiology for the first year of life and lung function and growth outcomes were summarized for ages 6-10 years. Results: From 2010-2020, there were 8,765 children with diagnosis of CF or CRMS entered into the CFFPR with sufficient diagnostic data for classification, of which 7,591 children had a clinical diagnosis of CF and 1,174 had a clinical diagnosis of CRMS. CRMS patients exhibited normal nutritional indices and pulmonary function up to age 9-10 years. The presence of respiratory bacteria associated with CF, such as Pseudomonas aeruginosa from CRMS patients ranged from 2.1-9.1% after the first year of life. Conclusions: Children with CRMS demonstrate normal pulmonary and nutritional outcomes into school age. However, a small percentage of children continue to culture CF-associated respiratory pathogens after infancy.
19 Jun 2023Submitted to Pediatric Pulmonology
19 Jun 2023Assigned to Editor
19 Jun 2023Submission Checks Completed
19 Jun 2023Review(s) Completed, Editorial Evaluation Pending
25 Jun 2023Reviewer(s) Assigned
19 Jul 2023Editorial Decision: Revise Major