title: Sickle-cell disease in Malawian children is associated with restrictive spirometry: a cross-sectional survey.
abstract: BACKGROUND
  A proportion of children with sickle-cell disease (SCD) demonstrate clinical findings consistent with the diagnosis of asthma. These children are at increased risk of complications, including acute chest syndrome.
  
  
  OBJECTIVE
  To assess lung function and symptoms of asthma in children with SCD in Blantyre, Malawi.
  
  
  METHODS
  Twenty-five children aged 7-16 years with electrophoretically confirmed SCD were recruited to undergo spirometry and questionnaire screening of asthma symptoms. Forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC) and FEV1/FVC ratio were compared with local and international reference ranges. Symptoms were assessed using the International Study of Asthma and Allergies in Childhood questionnaire.
  
  
  RESULTS
  Mean spirometric indices, represented as Z-scores derived from international reference ranges, were low: FEV1 -1.64 (95%CI -2.04 to -1.23), FVC -1.49 (95%CI -1.90 to -1.09), FEV1/FVC -0.39 (95%CI -0.76 to -0.03). Comparison with local reference ranges, represented as percentage of predicted value, revealed similar impairments: FEV1 86.9 (95%CI 81.1 to 92.7), FVC 89.0 (95%CI 83.5 to 94.4), FEV1/FVC ratio 97.7 (95%CI 95.4 to 99.9). The prevalence of wheeze was 16.7%.
  
  
  CONCLUSIONS
  We present spirometric abnormalities suggestive of restrictive lung disease with no evidence of obstructive defects or increased prevalence of wheeze.
authors.affiliation: Lewisham Hospital National Health Service Trust, London, UK. jamescook@doctors.org.uk
authors.forename: J
authors.initials: J
authors.lastname: Cook
authors.forename: O
authors.initials: O
authors.lastname: Jefferis
authors.forename: P
authors.initials: P
authors.lastname: Matchere
authors.forename: E
authors.initials: E
authors.lastname: Mbale
authors.forename: J
authors.initials: J
authors.lastname: Rylance
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000453:epidemiology; D001249:Asthma / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D002138:Calibration; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D005541:Forced Expiratory Volume; D019538:Health Care Surveys; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008295:Malawi / Q000453:epidemiology; D008297:Male; D011237:Predictive Value of Tests; D015995:Prevalence; D012015:Reference Standards; D012135:Respiratory Sounds / Q000175:diagnosis / Q000503:physiopathology; D013147:Spirometry / Q000592:standards; D011795:Surveys and Questionnaires; D014797:Vital Capacity