Global disparities in cystic fibrosis outcomes prior to CFTR modulators : a CF registries cohort study in South Africa and Canada

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dc.contributor.author Zampoli, Marco
dc.contributor.author Sykes, Jenna
dc.contributor.author Verstraete, Janine
dc.contributor.author Cheng, Stephanie Y.
dc.contributor.author Morrow, Brenda
dc.contributor.author Pepper, Michael Sean
dc.contributor.author Stewart, Cheryl
dc.contributor.author Zar, Heather J.
dc.contributor.author Stephenson, Anne L.
dc.date.accessioned 2024-08-30T08:36:20Z
dc.date.available 2024-08-30T08:36:20Z
dc.date.issued 2024-03
dc.description.abstract BACKGROUND : 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. METHODS : 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/m2 in adults. Standardised mean difference (SMD) >10 was considered significant. RESULTS : 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. CONCLUSION : 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. en_US
dc.description.department Immunology en_US
dc.description.librarian hj2024 en_US
dc.description.sdg SDG-03:Good heatlh and well-being en_US
dc.description.sponsorship The Canadian and SA CF Registry Steering Committees and registry collaborators, the Cystic Fibrosis Foundation, the SA CF Association, the Harry Crossley Foundation, the National Research Foundation of South Africa and South African Medical Research Council. en_US
dc.description.uri https://www.elsevier.com/locate/jcf en_US
dc.identifier.citation Zampoli, M., Sykes, J., Verstraete, J. et al. 2024, 'Global disparities in cystic fibrosis outcomes prior to CFTR modulators : a CF registries cohort study in South Africa and Canada', Journal of Cystic Fibrosis, vol. 23, no. 2, pp. 334-340, doi : 10.1016/j.jcf.2023.09.003. en_US
dc.identifier.issn 1569-1993 (print)
dc.identifier.issn 1873-501 (online)
dc.identifier.other 10.1016/j.jcf.2023.09.003
dc.identifier.uri http://hdl.handle.net/2263/97938
dc.language.iso en en_US
dc.publisher Elsevier en_US
dc.rights © 2023 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. This is an open access article under the CC BY-NC-ND license. en_US
dc.subject Cystic fibrosis en_US
dc.subject Low- and middle-income countries (LMICs) en_US
dc.subject South Africa (SA) en_US
dc.subject Canada en_US
dc.subject High income countries (HIC) en_US
dc.subject Cystic fibrosis transmembrane conductance regulator (CFTR) en_US
dc.subject Outcome disparities en_US
dc.subject SDG-03: Good health and well-being en_US
dc.title Global disparities in cystic fibrosis outcomes prior to CFTR modulators : a CF registries cohort study in South Africa and Canada en_US
dc.type Article en_US


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