Cystic fibrosis in South Africa: spectrum of disease and determinants of outcome.
Journal
ERJ open research
ISSN: 2312-0541
Titre abrégé: ERJ Open Res
Pays: England
ID NLM: 101671641
Informations de publication
Date de publication:
Jul 2021
Jul 2021
Historique:
received:
15
11
2020
accepted:
14
05
2021
entrez:
5
8
2021
pubmed:
6
8
2021
medline:
6
8
2021
Statut:
epublish
Résumé
Little is known about cystic fibrosis (CF) in low- to 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). Demographic, diagnosis and clinical data were extracted from the SACFR. Cross-sectional univariable and multivariable regression analysis of best forced expiratory volume in 1 s (FEV 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 ( 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.
Identifiants
pubmed: 34350279
doi: 10.1183/23120541.00856-2020
pii: 00856-2020
pmc: PMC8326682
pii:
doi:
Types de publication
Journal Article
Langues
eng
Informations de copyright
Copyright ©The authors 2021.
Déclaration de conflit d'intérêts
Conflict of interest: M. Zampoli has nothing to disclose. Conflict of interest: J. Verstraete has nothing to disclose. Conflict of interest: M. Frauendorf has nothing to disclose. Conflict of interest: R. Kassanjee has nothing to disclose. Conflict of interest: L. Workman has nothing to disclose. Conflict of interest: B.M. Morrow has nothing to disclose. Conflict of interest: H.J. Zar has nothing to disclose.
Références
Ann Intern Med. 2017 Apr 18;166(8):537-546
pubmed: 28288488
J Cyst Fibros. 2003 Mar;2(1):29-34
pubmed: 15463843
Pediatr Pulmonol. 2019 Sep;54(9):1382-1390
pubmed: 31144477
Pediatr Pulmonol. 2017 Nov;52(S48):S44-S51
pubmed: 28910520
J Cyst Fibros. 2013 Jul;12(4):363-6
pubmed: 23206872
S Afr Med J. 2006 Apr;96(4):304, 306
pubmed: 16670800
Pediatr Pulmonol. 2020 Jun;55(6):1375-1380
pubmed: 31338968
Curr Opin Pulm Med. 2018 Nov;24(6):574-578
pubmed: 30281026
J Med Genet. 1999 Jan;36(1):41-4
pubmed: 9950364
Paediatr Respir Rev. 2016 Sep;20:55-66
pubmed: 27259460
Am J Respir Crit Care Med. 2018 Oct 15;198(8):1055-1063
pubmed: 29742360
Genet Med. 2016 Jul;18(7):653-62
pubmed: 26656651
J Cyst Fibros. 2014 Sep;13(5):528-33
pubmed: 24485874
S Afr Med J. 2003 Jul;93(7):518-9
pubmed: 12939925
Pediatr Pulmonol. 2020 Jan;55(1):24-26
pubmed: 31531964
S Afr Med J. 1967 May 13;41(19):482-5
pubmed: 6027893
Paediatr Respir Rev. 2021 Jun;38:37-44
pubmed: 32826173
Pediatr Pulmonol. 2019 Apr;54(4):393-398
pubmed: 30656856
Sudan J Paediatr. 2014;14(1):39-44
pubmed: 27493388
Chest. 2009 May;135(5):1233-1242
pubmed: 19017867
Lancet Respir Med. 2013 Apr;1(2):121-8
pubmed: 24429092
J Cyst Fibros. 2021 May;20(3):473-484
pubmed: 32819855
S Afr Med J. 2018 Dec 13;109(1):16-19
pubmed: 30606298
J Cyst Fibros. 2018 May;17(3):297-305
pubmed: 29275954
S Afr Med J. 2006 Apr;96(4):288-9
pubmed: 16670795
Eur Respir J. 2014 Feb;43(2):505-12
pubmed: 23988764
J Cyst Fibros. 2020 Jul;19(4):540-545
pubmed: 31678015
Eur Respir J. 2012 Dec;40(6):1324-43
pubmed: 22743675
Am J Respir Crit Care Med. 2018 Mar 15;197(6):768-775
pubmed: 29099606
J Pediatr. 2017 Feb;181S:S4-S15.e1
pubmed: 28129811
Pediatr Pulmonol. 2017 Jul;52(7):909-915
pubmed: 28436621