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

• Published in: The international journal of tuberculosis and lung disease Vol. 17; no. 9; pp. 1235 - 1238
• Main Authors: Cook, J., Jefferis, O., Matchere, P., Mbale, E., Rylance, J.
• Format: Journal Article
• Language: English
• Published: Paris, France International Union Against Tuberculosis and Lung Disease 01.09.2013; International Union against Tuberculosis and Lung Disease
• Subjects: Adolescent; Allergies; Anemia, Sickle Cell - diagnosis; Anemia, Sickle Cell - epidemiology; Anemias. Hemoglobinopathies; Asthma; Asthma - diagnosis; Asthma - epidemiology; Asthma - physiopathology; Bacterial diseases; Biological and medical sciences; Calibration; Child; Chronic obstructive pulmonary disease, asthma; Cross-Sectional Studies; Diseases of red blood cells; Female; Forced Expiratory Volume; Health Care Surveys; Hematologic and hematopoietic diseases; Human bacterial diseases; Humans; Infectious diseases; Investigative techniques of respiratory function; Investigative techniques, diagnostic techniques (general aspects); Lung - physiopathology; Lung Function; Malawi - epidemiology; Male; Medical sciences; Mycobacterium; Paediatric; Pneumology; Predictive Value of Tests; Prevalence; Reference Standards; Respiratory Sounds - diagnosis; Respiratory Sounds - physiopathology; Spirometry - standards; Surveys and Questionnaires; Tuberculosis and atypical mycobacterial infections; Vital Capacity; Wheeze; Malawi; Africa; Human; Hemoglobinopathy; Lung disease; Pediatrics; Spirometry; Sickle cell anemia; Respiratory disease; wheeze; Exploration; Wheezing; Hemopathy; Genetic disease; Asthma; paediatric; Survey; Hemolytic anemia; Association; Lung function; Surveillance; Cross sectional study; Bronchus disease; Obstructive pulmonary disease; Child
• ISSN: 1027-3719; 1815-7920
• DOI: 10.5588/ijtld.12.0965

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.DESIGN: 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%.CONCLUSION: We present spirometric abnormalities suggestive of restrictive lung disease with no evidence of obstructive defects or increased prevalence of wheeze.