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.

中文翻译：

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西非和欧洲镰状细胞性贫血儿童肺功能差异

简介 肺功能异常在镰状细胞性贫血 (SCA) 中很常见，但来自撒哈拉以南非洲的数据有限。我们假设来自西非的 SCA 儿童的肺功能比来自欧洲的同龄人差。方法 这项前瞻性横断面研究评估了来自尼日利亚和英国、年龄在 6-18 岁、临床稳定的非洲黑人 SCA（血红蛋白表型 SS）个体的肺活量测定和人体测量。尼日利亚还包括年龄匹配的对照，以验证全球肺倡议肺活量测定参考值。结果 尼日利亚 SCA 患者 (n=154) 的 FEV1 和 FVC 与局部对照 (n=364) 相比显着降低了 ~1 z 分数，与英国患者 (n=101) 相比，z 分数显着降低了 ~0.5。消瘦（体重指数 z-score< -2) 在尼日利亚患者中的患病率为 27%，在英国患者中为 7%（p<0.001）。在患有 SCA 的儿童中，居住在尼日利亚（OR 2.4，95% CI 1.1 至 4.9）、消瘦（OR 2.3，95% CI 1.1 至 5.0）和每增加一岁（OR 1.2，95% CI 1.1 至 1.4）与限制性肺活量测定的风险增加独立相关（FVC z-score<-1.64+FEV1/FVC≥-1.64）。结论 本研究表明，西非 SCA 儿童的慢性呼吸障碍比欧洲更严重。我们的研究结果表明，对患有 SCA 的非洲儿童实施呼吸系统评估有助于及早识别那些患有慢性肺损伤的儿童，这些儿童有资格接受更密切的随访和更积极的治疗。1 至 5.0）和每增加一岁（OR 1.2，95% CI 1.1 至 1.4）与限制性肺活量测定的风险增加独立相关（FVC z 评分<-1.64+FEV1/FVC≥-1.64）。结论 本研究表明，西非 SCA 儿童的慢性呼吸障碍比欧洲更严重。我们的研究结果表明，对患有 SCA 的非洲儿童实施呼吸系统评估有助于及早识别那些患有慢性肺损伤的儿童，这些儿童有资格接受更密切的随访和更积极的治疗。1 至 5.0）和每增加一岁（OR 1.2，95% CI 1.1 至 1.4）与限制性肺活量测定的风险增加独立相关（FVC z 评分<-1.64+FEV1/FVC≥-1.64）。结论 本研究表明，西非 SCA 儿童的慢性呼吸障碍比欧洲更严重。我们的研究结果表明，对患有 SCA 的非洲儿童实施呼吸系统评估有助于及早识别那些患有慢性肺损伤的儿童，这些儿童有资格接受更密切的随访和更积极的治疗。