Original articles

Primary immunodeficiency in Africa – a review

A Erjaee, M Bagherpour, C van Rooyen, S van den Berg, C J Kinnear, R J Green, M Pepper

Abstract


Background. Efforts have been made worldwide to improve awareness and treatment of primary immunodeficiency (PID). This has also gained momentum on the African continent albeit at a slower pace. 

Objective. This review reports on the current status of PID on the African continent regarding its prevalence, distribution, genetic mutations and challenges in diagnosis and treatment of affected patients. 

Method. We evaluated all studies published from the African continent in the field of PID dealing with prevalence, epidemiology, case reports and genetic findings. 

Results. The prevalence of PID on the African continent has been estimated to be as high as 902 631 individuals. PID still is mostly underdiagnosed in Africa and although progress has been made in parts of the continent many challenges still remain regarding awareness, diagnosis, registration and care of these patients. 

Conclusion. Given the unique genetic mutations reported in PID patients on the African continent and the feasibility of hematopoietic stem cell transplantation and gene therapy, increased awareness should be encouraged and new therapeutic options considered.


Authors' affiliations

A Erjaee, Department of Immunology, Institute for Cellular and Molecular Medicine and SAMRC Extramural Unit for Stem Cell Research and Therapy, Faculty of Health Sciences, University of Pretoria, South Africa; Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran

M Bagherpour, Department of Immunology, Institute for Cellular and Molecular Medicine and SAMRC Extramural Unit for Stem Cell Research and Therapy, Faculty of Health Sciences, University of Pretoria, South Africa;Department of Industrial Engineering, Iran University of Science and Technology, Tehran, Iran

C van Rooyen, Department of Immunology, Ampath Pathology National Reference Laboratory, Pretoria, South Africa; Department of Paediatrics and Child Health, Steve Biko Academic Hospital and Faculty of Health Sciences, University of Pretoria, South Africa

S van den Berg, Department of Immunology, Ampath Pathology National Reference Laboratory, Pretoria, South Africa; Department of Paediatrics and Child Health, Steve Biko Academic Hospital and Faculty of Health Sciences, University of Pretoria, South Africa

C J Kinnear, DST-NRF Centre of Excellence for Biomedical Tuberculosis Research, South African Medical Research Council Centre for Tuberculosis Research, Division of Molecular Biology and Human Genetics, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa

R J Green, Department of Paediatrics and Child Health, Steve Biko Academic Hospital and Faculty of Health Sciences, University of Pretoria, South Africa

M Pepper, Department of Immunology, Institute for Cellular and Molecular Medicine and SAMRC Extramural Unit for Stem Cell Research and Therapy, Faculty of Health Sciences, University of Pretoria, South Africa

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Cite this article

South African Medical Journal 2019;109(8b):3-11. DOI:10.7196/SAMJ.2019.v109i8b.13820

Article History

Date submitted: 2019-09-10
Date published: 2019-09-10

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