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Sickle-cell disease in Malawian children is associated with restrictive spirometry: a cross-sectional survey

J Cook1, O Jefferis, P Matchere

  • 1Lewisham Hospital National Health Service Trust, London, UK. jamescook@doctors.org.uk

The International Journal of Tuberculosis and Lung Disease : the Official Journal of the International Union Against Tuberculosis and Lung Disease
|July 6, 2013
PubMed

Insights

Children with sickle-cell disease (SCD) often show lung function impairments suggestive of restrictive lung disease, not asthma. This study in Malawi assessed lung function and asthma symptoms in pediatric SCD patients.

Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Global Health

Background:

  • Sickle-cell disease (SCD) in children can present with asthma-like symptoms, increasing complication risks like acute chest syndrome.
  • Understanding lung function in pediatric SCD is crucial for managing associated respiratory risks.

Purpose of the Study:

  • To evaluate lung function and asthma symptoms in children with SCD in Blantyre, Malawi.
  • To identify potential restrictive or obstructive lung patterns in this population.

Main Methods:

  • Spirometry and asthma symptom questionnaires were administered to 25 children (aged 7-16) with confirmed SCD.
  • Lung function parameters (FEV1, FVC, FEV1/FVC) were compared to local and international reference ranges.
  • The International Study of Asthma and Allergies in Childhood questionnaire was used for symptom assessment.

Main Results:

  • Spirometry revealed low mean Z-scores for FEV1 (-1.64) and FVC (-1.49), indicating impaired lung function.
  • Comparison with local predicted values showed similar impairments (FEV1 86.9%, FVC 89.0%).
  • The prevalence of wheeze was 16.7%, with no significant obstructive defects identified.

Conclusions:

  • Spirometric abnormalities in children with SCD suggest restrictive lung disease.
  • No clear evidence of obstructive lung disease or an increased prevalence of wheeze was found in this cohort.
  • Further research into the specific patterns of lung disease in pediatric SCD is warranted.
Abstract

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