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Airways obstruction and pulmonary capillary blood volume in children with sickle cell disease

Catherine J Wedderburn1, David Rees, Susan Height

  • 1Division of Asthma, Allergy and Lung Biology, MRC & Asthma UK Centre in Allergic Mechanisms of Asthma, King's College London, London, SE5 9RS, United Kingdom.

Pediatric Pulmonology
|July 10, 2013
PubMed

Insights

In children with sickle cell disease (SCD), increased pulmonary capillary blood volume contributes to airway obstruction. This suggests bronchodilators may offer limited benefit for treating airway obstruction in these patients.

Area of Science:

  • Pulmonary Medicine
  • Pediatric Hematology
  • Respiratory Physiology

Background:

  • Airway obstruction is a significant complication in children with sickle cell disease (SCD).
  • Understanding the underlying physiological mechanisms is crucial for effective treatment strategies.

Purpose of the Study:

  • To investigate the relationship between pulmonary capillary blood volume and airway obstruction in children with SCD.
  • To determine if increased pulmonary capillary blood volume contributes to airway obstruction in pediatric SCD patients.

Main Methods:

  • An observational study comparing 25 children with SCD to 25 matched controls.
  • Assessment of respiratory system resistance (R5 %pred), pulmonary capillary blood volume (Vc), alveolar volume (VA), and spirometry before and after bronchodilator administration.
  • Lung volume measurements were also performed.

Main Results:

  • Children with SCD exhibited higher respiratory system resistance and pulmonary capillary blood volume compared to controls, both before and after bronchodilator use.
  • Pulmonary capillary blood volume (Vc/VA) showed a significant correlation with respiratory system resistance (R5 %pred) specifically in children with SCD.
  • Bronchodilator administration resulted in similar reductions in respiratory system resistance in both groups, with no significant changes in pulmonary capillary blood volume.

Conclusions:

  • Increased pulmonary capillary blood volume is a contributing factor to airway obstruction in children with SCD.
  • The findings suggest that bronchodilators may have limited efficacy in ameliorating airway obstruction in this population.
  • Further research into alternative therapeutic approaches targeting pulmonary vascular changes in pediatric SCD is warranted.
Abstract

Keywords:
anemiaimpulse oscillometrylung function

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