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Growth and long-term lung function in cystic fibrosis: a longitudinal study of patients diagnosed by neonatal

Baroukh Maurice Assael1, Giovanni Casazza, Patrizia Iansa

  • 1Cystic Fibrosis Center, Verona, Italy. baroukh.assael@unimi.it

Pediatric Pulmonology
|February 21, 2009
PubMed

Insights

Cystic fibrosis (CF) patients with severe lung disease show delayed growth milestones and reduced peak height velocity from childhood. These growth issues appear early, even before significant pulmonary function decline in CF patients.

Area of Science:

  • Pediatric Endocrinology
  • Pulmonology
  • Genetics

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
  • Long-term growth patterns and their relationship to respiratory function in CF are not fully understood.
  • Early identification of growth deviations is crucial for managing CF patients.

Purpose of the Study:

  • To analyze the relationship between growth milestones and respiratory function in cystic fibrosis patients from childhood to adulthood.
  • To evaluate linear growth and lung disease severity in CF.
  • To establish a correlation between growth and disease severity throughout childhood.

Main Methods:

  • A cohort of 163 CF patients with complete follow-up data until age 20 was analyzed.
  • Growth profiles were modeled using a nonlinear function, and multivariate analysis assessed growth milestones against disease severity.
  • Patient data were compared to the normal Italian population, with disease severity classified by FEV1.

Main Results:

  • Lung disease severity was found to correlate with delayed prepubertal and pubertal growth milestones in CF patients.
  • Peak height velocities were significantly reduced in patients with more severe lung disease.
  • Growth abnormalities were observed early in severe CF cases, preceding notable pulmonary decline.

Conclusions:

  • Severe cystic fibrosis is associated with reduced growth velocity at an early age.
  • Growth milestones are significantly impacted by lung disease severity in CF.
  • Monitoring growth is essential for early detection of disease progression in pediatric CF.
Abstract

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