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Related Experiment Videos

Lung function abnormalities in cystic fibrosis and changes during growth.

A Zapletal, J Houstek, M Samanek

    Bulletin Europeen De Physiopathologie Respiratoire
    |July 1, 1979
    PubMed
    Summary

    This study found that maximal expiratory flows (Vmax) and upstream airway conductance (Gus) were most abnormal in cystic fibrosis patients. Lung function generally declined with growth, but specific airway parameters showed little change over time.

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    Area of Science:

    • Pulmonary Medicine
    • Pediatric Pulmonology
    • Respiratory Physiology

    Background:

    • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
    • Pulmonary complications, including airflow obstruction and reduced lung elasticity, are hallmarks of CF.
    • Longitudinal assessment of lung function in pediatric CF patients is crucial for understanding disease progression.

    Purpose of the Study:

    • To longitudinally evaluate various lung function parameters in pediatric cystic fibrosis patients.
    • To identify the most consistently abnormal indices of airway function and lung elasticity in CF.
    • To assess the impact of growth and time on lung function in this cohort.

    Main Methods:

    • Studied 28 pediatric patients (5-25 years) with CF over 1-5 years.

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  • Measured static lung volumes, elastic recoil pressure, lung compliance, airway conductance, FEV1, and maximal expiratory flows.
  • Compared data to normal values, used regression equations related to body height, and expressed results as percentages of predicted values.

    • Main Results:

    • Maximal expiratory flows (Vmax) at low lung volumes, upstream airway conductance (Gus) at 60% TLC, residual volume (RV), and RV/TLC were the most consistently abnormal airway function parameters.
    • Pulmonary elastic recoil pressure (Pst(1)) at 60% TLC was the most abnormal index of lung elasticity.
    • Most lung function indices (VC, TLC, FRC, Pst(1)) generally declined with growth, while Vmax and Gus did not worsen, likely due to pre-existing abnormalities.

    Conclusions:

    • Airway obstruction and reduced lung elasticity are significant in pediatric CF, with Vmax and Gus being particularly affected.
    • Lung function shows a general decline with growth, but certain airway parameters remain stable, possibly indicating early-onset abnormalities.
    • Lung function stability over 1-5 years (approx. 10 cm height increase) was observed in over 50% of patients, with some improving and others deteriorating.