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Related Experiment Videos

Pulmonary function abnormalities in childhood sickle cell disease

P Pianosi1, S J D'Souza, T D Charge

  • 1Respiratory Medicine Service, Montreal Children's Hospital, Quebec, Canada.

The Journal of Pediatrics
|March 1, 1993
PubMed
Summary

Children with sickle cell anemia, especially HbSS, often have smaller lungs. Pulmonary function tests reveal reduced lung volumes, not airway obstruction, regardless of acute chest syndrome history.

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Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is linked to restrictive pulmonary patterns in adults, often attributed to acute chest syndrome (ACS).
  • Limited data exists on pulmonary function in pediatric SCD populations, particularly concerning the impact of ACS.
  • Understanding lung abnormalities in children with SCD is crucial for early intervention and management.

Purpose of the Study:

  • To compare pulmonary function test (PFT) results in children with sickle cell anemia (SCA) and healthy controls.
  • To investigate the association between pulmonary function and a history of acute chest syndrome (ACS) in pediatric SCA patients.
  • To determine if children with SCA exhibit restrictive lung patterns and identify underlying causes.

Main Methods:

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  • Pulmonary function tests (PFTs) including forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), diffusion capacity of carbon monoxide, and lung volumes were performed.
  • 37 children with SCA (HbSS, HbSC, S beta-thalassemia) were compared to 22 matched control subjects.
  • Patients were stratified based on a history of ACS.

    • Main Results:

    • Children with HbSS demonstrated significantly reduced FVC and FEV1 compared to controls, irrespective of ACS history (p < 0.05).
    • Total lung capacity was also significantly lower in HbSS patients versus controls (p < 0.05).
    • Airway obstruction indices (FEV1/FVC ratio, residual volume/total lung capacity ratio) were normal in all pediatric SCA groups.

    Conclusions:

    • Children with sickle cell anemia, particularly HbSS, exhibit abnormally small lungs with normal function relative to size.
    • The observed lung abnormalities in pediatric SCA are not solely attributable to acute chest syndrome.
    • These findings suggest intrinsic factors may contribute to reduced lung volumes in pediatric SCD.