Journal of Clinical Immunology

, Volume 31, Issue 1, pp 99–105

Primary Immunodeficiencies: A 27-Year Review at a Tertiary Paediatric Hospital in Cape Town, South Africa

  • Reené Naidoo
  • Lisa Ungerer
  • Margaret Cooper
  • Sandra Pienaar
  • Brian S. Eley
Article

DOI: 10.1007/s10875-010-9465-7

Cite this article as:
Naidoo, R., Ungerer, L., Cooper, M. et al. J Clin Immunol (2011) 31: 99. doi:10.1007/s10875-010-9465-7

Abstract

Introduction

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.

Methods

A retrospective study was conducted on 168 patients diagnosed with PID from 1983 to 2009.

Results

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.

Conclusions

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.

Keywords

Primary immunodeficiencieschildrenSouth Africaepidemiology

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Reené Naidoo
    • 1
    • 3
  • Lisa Ungerer
    • 2
  • Margaret Cooper
    • 1
  • Sandra Pienaar
    • 1
  • Brian S. Eley
    • 1
  1. 1.Paediatric Infectious Diseases Unit, Red Cross War Memorial Children’s Hospital and the School of Child and Adolescent HealthUniversity of Cape TownCape TownSouth Africa
  2. 2.Department of PathologyRed Cross War Memorial Children’s Hospital, National Health Laboratory ServicesCape TownSouth Africa
  3. 3.Paediatric Infectious Diseases UnitRed Cross War Memorial Children’s HospitalCape TownSouth Africa