RT Journal Article
SR Electronic
T1 Cystic fibrosis in South Africa: spectrum of disease and determinants of outcome
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00856-2020
DO 10.1183/23120541.00856-2020
A1 M. Zampoli
A1 J. Verstraete
A1 M. Frauendorf
A1 R. Kassanjee
A1 L. Workman
A1 B. M. Morrow
A1 H. J. Zar
YR 2021
UL http://openres.ersjournals.com/content/early/2021/05/25/23120541.00856-2020.abstract
AB Introduction Little is known about cystic fibrosis (CF) in low-middle income settings. This study aimed to describe the spectrum and outcomes of CF in South Africa (SA) from the recently established SA CF registry (SACFR).Methods Demographic, diagnosis and clinical data was extracted from the SACFR. Cross-sectional univariable and multivariable regression analysis of best forced expiratory volume in 1 s (FEV1; age≥6 years) and nutrition (all ages) in 2018 was conducted to investigate factors associated with severe lung disease (SLD; FEV1 ≤3.0 z-score) and undernutrition.Results By December 2018, ancestry of 447 individuals included in the SACFR was Caucasian (315; 70%), mixed (87; 19%) and black African (41; 9%). Median diagnosis age was 7.6 months (IQR 2.7,37.1). Genotype was p.Phe508del homozygous (220; 49%); p.Phe508del heterozygous (144; 32%) and neither p.Phe508del or unknown Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variant in 83 (19%); the second most frequent CFTR variant was 3120+1G>A, common in black Africans. Median age of patients in 2018 was 14.7 years (IQR 7.4,24.4). SLD was independently associated with chronic methicillin resistant S.aureus (MRSA) (aOR 16.75; 95% CI 1.74–161.50), undernutrition (aOR 5.20; 95% CI 2.23–12.13) and age (aOR 2.23 per 10-years; 95% CI 1.50–3.31). Undernutrition was associated in univariable analysis with low weight at diagnosis, non-caucasian ancestry, chronic P.aeruginosa infection and lower socioeconomic status.Conclusion Interventions targeting MRSA infection and nutrition are needed to improve CF outcomes in SA. Most people with CF in SA are eligible for highly effective CFTR modulator therapy.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Zampoli has nothing to disclose.Conflict of interest: Dr. Verstraete has nothing to disclose.Conflict of interest: Dr. Frauendorf has nothing to disclose.Conflict of interest: Dr. Kassanjee has nothing to disclose.Conflict of interest: Dr. Workman has nothing to disclose.Conflict of interest: Dr. Morrow has nothing to disclose.Conflict of interest: Dr. Zar has nothing to disclose.