Lung function in children with sickle cell disease from Central Africa

Abstract

Lung function in patients with sickle cell anaemia (SCA) living in sub-Saharan Africa is largely unknown. Anthropometry and spirometry were cross-sectionally evaluated in patients with SCA (HbSS) aged 6–18 years and in schoolchildren from the Democratic Republic of the Congo. The Global Lung Initiative 2012 spirometry reference values were used. A total of 112 patients and 377 controls were included. Twenty-six per cent of patients with SCA had spirometry findings suggestive of a restrictive pattern and 41% had a FEV1 z-score <5th percentile. Wasting, increasing age and female sex were independently associated with increased risk of restrictive spirometry pattern in patients with SCA. Longitudinal studies could clarify the prognostic meaning of these findings.

Differences in lung function between children with sickle cell anaemia from West Africa and Europe

“…8 13 Asthma was more frequent in the UK SCA group than in the Nigerian one. Asthma is reported in 15%-28% of people with SCA living in HICs, 31 whereas we found that less than 10% of SCA patients in the Democratic Republic of Congo (Central Africa) 13 and Nigeria (West Africa) had a physician's diagnosis of asthma. Some of these children could have been underdiagnosed, due to poor healthcare access in sub-Saharan Africa.…”

“…The results are in line with those of previous studies reporting a positive association between BMI percentile and FEV 1 , 14 as well as an increased risk of restrictive spirometry pattern in SCA patients with wasting. 13 There are several factors potentially implicated in the relationship between nutritional status and lung function in SCA patients. First of all, wasting could reflect increased metabolic demands due to a more severe course of disease, 38 including worse respiratory manifestations.…”

“…7 Regarding risk factors for abnormal lung function in paediatric SCA patients, some studies pointed out an association with a history of acute chest syndrome (ACS) 3 11 12 but other did not, [8][9][10] whereas the rate of pain crises seems to be unrelated to respiratory abnormalities. 8 10 Malnutrition is associated with increased risk of restrictive spirometry pattern in children with SCA from Central Africa, 13 while the body mass index (BMI) percentile was found to be an independent predictor of increase in FEV 1 in patients from HICs. 14 Although limited evidence is available, it seems that lung function abnormalities are highly prevalent in paediatric patients with SCA from sub-Saharan Africa, 12 13 15 16 with restrictive spirometry being the most frequent pathological pattern.…”

“…14 Although limited evidence is available, it seems that lung function abnormalities are highly prevalent in paediatric patients with SCA from sub-Saharan Africa, 12 13 15 16 with restrictive spirometry being the most frequent pathological pattern. 13 The burden of respiratory disease in children with SCA living in low-middle income countries (LMICs) and HICs has not been previously compared. This evaluation could be useful because, if substantial…”

Management of chronic respiratory complications in children and adolescents with sickle cell disease

“…In paediatric patients with SCD from high-income countries, an obstructive physiology (forced expiratory volume in 1 s (FEV 1 )/forced vital capacity (FVC) ratio <LLN (<5th percentile) and total lung capacity (TLC) ⩾LLN) is the most frequent lung function alteration, noted in approximately 15-20% of the cases [12,13]. In low-to middle-income countries, however, cross-sectional spirometry data in children with SCD aged 6-19 years showed that 25-30% of participants had reduced FEV 1 and FVC <5th percentile with preserved FEV 1 /FVC ratio, possibly indicating a restrictive (TLC <LLN) or nonspecific pattern (TLC ⩾LLN) [14,15]. Diffusing capacity (adjusted for haemoglobin values) can also be impaired in paediatric patients with SCD [13], more often in adolescents with a restrictive physiology [16].…”