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Differences in lung function between children with sickle cell anaemia from West Africa and Europe
Michele Arigliani1, Luigi Castriotta2, Ramatu Zubair3
1Department of Medicine, Division of Pediatrics, University Hospital of Udine, Udine, Italy michelearigliani@gmail.com.
Insights
Children with sickle cell anaemia (SCA) in West Africa experience more severe lung function abnormalities than those in Europe. Early respiratory assessments are crucial for identifying chronic lung injury in African children with SCA.
Area of Science:
- Pulmonary Medicine
- Hematology
- Pediatrics
Background:
- Lung function abnormalities are prevalent in sickle cell anaemia (SCA).
- Limited data exists on respiratory health in sub-Saharan African children with SCA.
- West African children with SCA may have worse lung function compared to European counterparts.
Purpose of the Study:
- To compare lung function in Nigerian and UK children with SCA.
- To investigate factors associated with restrictive spirometry in children with SCA.
Main Methods:
- Prospective cross-sectional study of SCA patients (hemoglobin phenotype SS) aged 6-18 years from Nigeria and the UK.
- Spirometry and anthropometry were evaluated in clinically stable patients.
- Age-matched controls were included in Nigeria to validate reference values.
Main Results:
- Nigerian SCA patients showed significant reductions in FEV1 and FVC compared to local controls and UK patients.
- Prevalence of wasting was higher in Nigerian SCA patients (27%) than in UK patients (7%).
- Living in Nigeria, wasting, and older age were independently associated with increased risk of restrictive spirometry.
Conclusions:
- Chronic respiratory impairment is more severe in West African children with SCA than in European children.
- Implementing respiratory assessment in African children with SCA can help identify chronic lung injury.
- Early identification allows for closer follow-up and more aggressive therapeutic interventions.
Introduction:
Lung function abnormalities are common in sickle cell anaemia (SCA) but data from sub-Saharan Africa are limited. We hypothesised that children with SCA from West Africa had worse lung function than their counterparts from Europe.
Methods:
This prospective cross-sectional study evaluated spirometry and anthropometry in black African individuals with SCA (haemoglobin phenotype SS) aged 6-18 years from Nigeria and the UK, when clinically stable. Age-matched controls were also included in Nigeria to validate the Global Lung Initiative spirometry reference values.
Results:
Nigerian SCA patients (n=154) had significant reductions in both FEV1 and FVC of ~1 z-score compared with local controls (n=364) and ~0.5 z-scores compared with the UK patients (n=101). Wasting (body mass index z-score<-2) had a prevalence of 27% in Nigerian patients and 7% in the UK ones (p<0.001). Among children with SCA, being resident in Nigeria (OR 2.4, 95% CI 1.1 to 4.9), wasting (OR 2.3, 95% CI 1.1 to 5.0) and each additional year of age (OR 1.2, 95% CI 1.1 to 1.4) were independently associated with increased risk of restrictive spirometry (FVC z-score<-1.64+FEV1/FVC≥-1.64).
Conclusions:
This study showed that chronic respiratory impairment is more severe in children with SCA from West Africa than Europe. Our findings suggest the utility of implementing respiratory assessment in African children with SCA to early identify those with chronic lung injury, eligible for closer follow-up and more aggressive therapies.
