Primary Immunodeficiencies: A 27-Year Review at a Tertiary Paediatric Hospital in Cape Town, South Africa
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The epidemiology of primary immunodeficiencies (PID) is not well documented in Africa. The objective of this study was to describe the spectrum of PID at a tertiary paediatric centre in South Africa.
A retrospective study was conducted on 168 patients diagnosed with PID from 1983 to 2009.
Over the study period, antibody deficiencies predominated (51%) followed by well-defined syndromes (24%). Common variable immunodeficiency was the commonest antibody deficiency. The mean age of diagnosis was 51 months overall but decreased significantly to 35 months over the last 9 years. Recurrent infections were the most common presenting complaint (74%). The overall mortality rate was 25% while combined immunodeficiencies accounted for 40% of the deaths.
The spectrum of PID in South Africa was similar to international trends. The declining mean age of diagnosis indicated improved recognition of PID. Future research should focus on identifying children with PID more effectively.
KeywordsPrimary immunodeficiencies children South Africa epidemiology
- 7.Benjasupattananan P, Simasathein T, Vichyanond P, Leungwedchakarn V, Visitsunthorn N, Pacharn P, et al. Clinical characteristics and outcomes of primary immunodeficiencies in Thai children: an 18 year experience from a tertiary care center. J Clin Immunol. 2009;29:357–64.CrossRefPubMedGoogle Scholar
- 14.Eley BS, Hughes EJ, Cooper M, Pienaar S, Shaboodien G, Henderson H, Beatty DW. Primary immunodeficiency diseases in Cape Town. Abstract: K2.Mon.1.21/1; 11th International Congress of Immunology. Stockholm, Sweden, July 2001.Google Scholar
- 15.Pienaar S, Eley BS, Hughes J, Henderson HE. X-linked hyper IgM (HIGM1) in an African kindred: the first report from South Africa. BMC Pediatr. 2003;3(12).Google Scholar
- 18.Statistics South Africa: mid-year population estimates 2010. www.statssa.gov.sa (2010). Accessed 16 August 2010.