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Observational Study
. 2022 Apr;57(4):908-918.
doi: 10.1002/ppul.25810. Epub 2022 Jan 11.

Trends in cystic fibrosis survival over 40 years in South Africa: An observational cohort study

Marco Zampoli  1   2 , Reshma Kassanjee  3 , Janine Verstraete  1 , Anthony Westwood  1 , Heather J Zar  1   2 , Brenda M Morrow  1
Affiliations
Observational Study

Trends in cystic fibrosis survival over 40 years in South Africa: An observational cohort study

Marco Zampoli et al. Pediatr Pulmonol. 2022 Apr.

Abstract

Introduction: Temporal trends in cystic fibrosis (CF) survival from low-middle-income settings is poorly reported. We describe changes in CF survival after diagnosis over 40 years from a South African (SA) CF center.

Methods: An observational cohort study of people diagnosed with CF from 1974 to 2019. Changes in age-specific mortality rates from 2000 (vs. before 2000) were estimated using multivariable Poisson regression. Data were stratified by current age < or ≥10 years and models controlled for diagnosis age, sex, ethnicity, genotype, and Pseudomonas aeruginosa (PA) infection. A second analysis explored the association of mortality with weight and forced expiratory volume in 1 s reported as z-scores (FEV1z-scores) at age 5-8 years.

Results: A total of 288 people (52% male; 57% Caucasian; 44% p.Phe508del homozygous) were included (median diagnosis age 0.5 years: Q1, Q3: 0.2, 2.5); 100 (35%) died and 30 (10%) lost to follow-up. Among age >10 years, age-specific mortality from 2000 was significantly lower (adjusted hazard ratio [aHR]: 0.14; 95% confidence interval [CI]: 0.06, 0.29; p < 0.001), but not among age <10 years (aHR: 0.67; 95% CI: 0.28, 1.64; p = 0.383). In children <10 years, Caucasian ethnicity was associated with lower mortality (aHR 0.17; 95% CI: 0.05, 0.63), and longer times since first PA infection with higher mortality (aHR: 1.31; 95% CI: 1.01, 1.68). Mortality was sevenfold higher if FEV1z was <-2.0 at age 5-8 years (aHR: 7.64; 95% CI: 2.58, 22.59).

Conclusion: Overall, CF survival has significantly improved in SA from 2000 in people older than 10 years. However, increased risk of mortality persists in young non-Caucasian children, and with FEV1z <-2.0 at age 5-8 years.

Keywords: South Africa; cystic fibrosis; low-middle income; survival.

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References

REFERENCES

    1. Corriveau S, Sykes J, Stephenson AL. Cystic fibrosis survival: the changing epidemiology. Curr Opin Pulm Med. 2018;24(6):574-578. doi:10.1097/MCP.0000000000000520
    1. Stephenson AL, Sykes J, Stanojevic S, et al. Survival comparison of patients with cystic fibrosis in Canada and the United States: a population-based cohort study. Ann Intern Med. 2017;166(8):537-546. doi:10.7326/M16-0858
    1. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation annual patient registry report 2019. 2019. Accessed March 26, 2021. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2019-...
    1. Jackson AD, Goss CH. Epidemiology of CF: how registries can be used to advance our understanding of the CF population. J Cyst Fibros. 2018;17(3):297-305. doi:10.1016/j.jcf.2017.11.013
    1. Keogh RH, Stanojevic S. A guide to interpreting estimated median age of survival in cystic fibrosis patient registry reports. J Cyst Fibros. 2018;17(2):213-217. doi:10.1016/j.jcf.2017.11.014

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