Department of Paediatrics and Child Health, University of Cape Town, South Africa; South African MRC Unit for Child and Adolescent Health, University of Cape Town, South Africa.
Department of Respirology, Adult Cystic Fibrosis Program, St. Michael's Hospital, University of Toronto, Toronto, Canada.
J Cyst Fibros. 2024 Mar;23(2):334-340. doi: 10.1016/j.jcf.2023.09.003. Epub 2023 Sep 12.
Outcomes of cystic fibrosis (CF) differ between low-middle income and high-income countries, but comparative data are lacking. We compared South African (SA) and Canadian CF outcomes to explore what disparities existed prior to access of CFTR modulators in Canada.
A cross-sectional study of SA and Canadian CF registries data for period 1 January to 31 December 2018. CF registry data were harmonised between countries to compare lung function and nutrition outcomes. Poor nutrition was defined as BMIz-score < -1 in children and < 18.5 kg/m in adults. Standardised mean difference (SMD) >10 was considered significant.
After excluding Canadians on CFTR modulators and lung transplant recipients, data on 4049 Canadian and 446 SA people was analysed. Compared to Canada, people in SA were younger (median age 15.8 years vs. 24.1 years: SMD 52) with fewer males (47.8% vs 54.2%; SMD 12.5) and White (70.9% vs. 93.3%; SMD 61.3). Class I-III CFTR mutation frequency was similar in SA (n = 384, 86.1%) and Canada (n = 3426, 84.9%). After adjusting for age, gender, diagnosis age, genotype, P.aeruginosa infection and pulmonary treatments, FEV1pp was 8.9% lower (95% CI 6.3% to 11.4%) and poor nutrition 1.7-fold more common (OR 1.70; 95% CI 1.19-2.41) in SA compared to Canada.
Lung function and nutrition was significantly lower in SA compared to Canada. Global disparities in CF outcomes between high and low-middle income countries are likely to widen as CFTR modulators are rapidly scaled up in only high-income countries.
囊性纤维化 (CF) 在中低收入国家和高收入国家的结局不同，但缺乏比较数据。我们比较了南非 (SA) 和加拿大 CF 的结局，以探索在加拿大获得 CFTR 调节剂之前存在哪些差异。
这是一项对南非和加拿大 CF 登记处数据的横断面研究，研究时间为 2018 年 1 月 1 日至 12 月 31 日。对两个国家的 CF 登记处数据进行了协调，以比较肺功能和营养结局。营养状况不良定义为儿童 BMIz 评分< -1 和成人<18.5 kg/m。SMD >10 被认为有显著差异。
排除接受 CFTR 调节剂治疗和肺移植的加拿大患者后，分析了 4049 名加拿大和 446 名南非患者的数据。与加拿大相比，南非患者年龄更小（中位数年龄 15.8 岁比 24.1 岁：SMD 52），男性比例更低（47.8%比 54.2%：SMD 12.5），白人比例更低（70.9%比 93.3%：SMD 61.3）。南非（n=384，86.1%）和加拿大（n=3426，84.9%）I-III 类 CFTR 突变频率相似。在调整年龄、性别、诊断年龄、基因型、铜绿假单胞菌感染和肺部治疗后，南非的 FEV1pp 低 8.9%（95%CI 6.3%至 11.4%），营养状况不良的比例高 1.7 倍（OR 1.70；95%CI 1.19 至 2.41）。
与加拿大相比，南非的肺功能和营养明显更差。随着 CFTR 调节剂仅在高收入国家迅速推广，高收入和中低收入国家之间 CF 结局的全球差异可能会进一步扩大。
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