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Lung function comparison between two decades in cystic fibrosis children: A single centre study

Gloria Tridello1, Sonia Volpi1, Baroukh M Assael1

  • 1Cystic Fibrosis Center, Verona, Italy.

Pediatric Pulmonology
|October 1, 2015
PubMed

Insights

This study found improved lung function in cystic fibrosis (CF) patients diagnosed via newborn screening over two decades. These findings highlight the impact of modern CF therapies on patient outcomes.

Area of Science:

  • Pulmonology
  • Pediatrics
  • Genetics

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Neonatal screening allows for early diagnosis and intervention.
  • Therapeutic advancements have significantly impacted CF patient care.

Purpose of the Study:

  • To compare pulmonary function decline in two CF patient cohorts born in different decades.
  • To assess the impact of neonatal screening and evolving treatments on CF outcomes.
  • To identify a potential cohort effect in CF patient lung function.

Main Methods:

  • Retrospective analysis of CF patients born between 1979-1984 (Cohort 1) and 1991-1996 (Cohort 2).
  • Comparison of pulmonary function decline (FEV1%, FEF 25-75%) from ages 10 to 15.
  • Statistical analysis using a linear mixed model approach to evaluate cohort differences.

Main Results:

  • Significant amelioration in pulmonary function decline observed in Cohort 2 compared to Cohort 1 (P < 0.001 for both FEV1% and FEF 25-75%).
  • Mean absolute decrement of FEV1% was 9.2 in Cohort 1 vs. 0.6 in Cohort 2.
  • Mean decrement of FEF 25-75% was 16.3 in Cohort 1 vs. 1.3 in Cohort 2.

Conclusions:

  • Pulmonary function has markedly improved in young CF patients over a decade.
  • This improvement correlates with the introduction of key therapies like dornase alfa, tobramycin, and hypertonic saline.
  • This study provides evidence of a cohort effect in CF patients diagnosed through neonatal screening.
Abstract

Keywords:
Cystic fibrosisFEV1adolescencenebulized antibiotics

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