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Lung function in infants with sickle cell disease

A C Koumbourlis1, A Hurlet-Jensen, M R Bye

  • 1Department of Pediatrics, College of Physicians and Surgeons of Columbia University, New York, New York, USA.

Pediatric Pulmonology
|November 22, 1997
PubMed
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Sickle cell disease can cause abnormal lung function, specifically lower airway obstruction and hyperinflation, even in early infancy. These early lung changes may be linked to airway reactivity but require further study.

Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Neonatology

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder with potential systemic complications.
  • Pulmonary complications in SCD are well-documented in older individuals but less understood in infants.
  • Early detection of lung dysfunction in infants with SCD is crucial for timely intervention.

Purpose of the Study:

  • To investigate the presence and nature of pulmonary function abnormalities in infants with sickle cell disease.
  • To determine if abnormal lung function develops early in life in this population.

Main Methods:

  • Pulmonary function testing was conducted on 20 infants (3-30 months) with SCD.
  • Techniques included passive occlusion for respiratory system compliance and resistance, nitrogen washout for functional residual capacity, and thoracoabdominal compression for tidal flow-volume loops.

Related Experiment Videos

  • Patients were categorized into Hb SS (Group I) and other hemoglobinopathies (Group II).

    • Main Results:

    • Both groups exhibited elevated functional residual capacity (FRC) and decreased maximum expiratory flows at FRC, indicating lower airway obstruction (LAO) and hyperinflation.
    • Patients with Hb SS showed a trend towards more acute chest syndrome (ACS) and vasoocclusive crisis (VOC).
    • Restrictive lung disease was uncommon, observed in only three patients.

    Conclusions:

    • Infants with sickle cell disease, particularly those with Hb SS, may present with abnormal lung function, predominantly LAO and hyperinflation, in early infancy.
    • Airway reactivity is a potential factor in the pathogenesis of these early pulmonary changes.
    • The precise relationship between these early lung abnormalities and clinical SCD events like VOC or ACS remains to be elucidated.