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

Autor: Zampoli M; 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. Electronic address: m.zampoli@uct.ac.za., Sykes J; Department of Respirology, Adult Cystic Fibrosis Program, St. Michael's Hospital, University of Toronto, Toronto, Canada., Verstraete J; Department of Paediatrics and Child Health, University of Cape Town, South Africa., Cheng SY; Cystic Fibrosis Canada, Toronto, Canada., Morrow B; Department of Paediatrics and Child Health, University of Cape Town, South Africa., Pepper MS; Department of Immunology, Institute for Cellular and Molecular Medicine, SAMRC Extramural Unit for Stem Cell Research and Therapy, University of Pretoria, South Africa., Stewart C; Department of Basic Medical Sciences, The University of the West Indies, Mona, Jamaica., Zar HJ; 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., Stephenson AL; Department of Respirology, Adult Cystic Fibrosis Program, St. Michael's Hospital, University of Toronto, Toronto, Canada.
Jazyk: angličtina
Zdroj: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2024 Mar; Vol. 23 (2), pp. 334-340. Date of Electronic Publication: 2023 Sep 12.
DOI: 10.1016/j.jcf.2023.09.003
Abstrakt: 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/m 2 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.
Competing Interests: Declaration of Competing Interest None.
(Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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