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Growth of lung function in children with sickle cell anemia

Joshua J Field1, Michael R DeBaun2, Yan Yan3

  • 1Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri.

Pediatric Pulmonology
|October 31, 2008
PubMed

Insights

Children with sickle cell disease (SCD) show reduced lung function growth compared to the general population. This study highlights potential gender differences in developing abnormal lung function and airway obstruction in pediatric SCD patients.

Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Growth and Development

Background:

  • Lung disease is a significant cause of illness in children with sickle cell disease (SCD).
  • Previous studies show abnormal pulmonary function in children with SCD, but lung function growth patterns remain unknown.
  • Understanding lung function growth is crucial for managing pediatric SCD.

Purpose of the Study:

  • To provide evidence that lung function growth is reduced in children with SCD.
  • To compare lung function growth in children with hemoglobin SS (HbSS) to a healthy control group.
  • To investigate potential gender-specific differences in lung function development in pediatric SCD.

Main Methods:

  • Retrospective cohort study of 79 children with HbSS (ages 6-19) with at least two spirometry assessments.
  • Comparison of lung function growth data with 255 age-, gender-, and race-matched controls from the Harvard Six Cities Study (H6CS).
  • Longitudinal analysis of spirometry parameters including forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC).

Main Results:

  • Children with HbSS exhibited lower longitudinal FEV1 compared to the general population (P = 0.031 for boys, P = 0.002 for girls).
  • Girls with HbSS showed significantly lower longitudinal FVC (P < 0.001) and FEV1/FVC ratio (P = 0.038) compared to H6CS controls.
  • No significant difference in FVC or FEV1/FVC was observed between boys with HbSS and H6CS controls.

Conclusions:

  • Lung function growth is attenuated in children with HbSS compared to healthy peers.
  • Gender may play a role in the risk of abnormal lung function and airway obstruction in pediatric SCD.
  • Further research is warranted to explore gender-specific pulmonary complications in pediatric SCD.

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