ArticleSouth African family practice : official journal of the South African Academy of Family Practice/Primary Care2022
The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng.
Article in South African family practice : official journal of the South African Academy of Family Practice/Primary Care, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 2 of them syntheses that pooled it.
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Who cites it
2 citing papers in PubMed, 2 syntheses or guidelines pooled it, 1 citations in OpenAlex.
- Advancing genetic counselling in Southern Africa: Unveiling opportunities for inclusive healthcare and genomic education for Angola.Saudi medical journal · 2025Pooled it
- The prevalence of Hemophilia A in males in Africa: evidence from a systematic review and meta-analysis.BMC public health · 2024Pooled it
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHaemophilia A and B are X-linked recessive bleeding disorders resulting from a deficiency of factors VIII and IX, respectively. Early diagnosis and a comprehensive approach to management is mandatory. This study aimed to describe the profile of patients with haemophilia (PWH) managed at Dr George Mukhari Academic Hospital (DGMAH) with the view to identify potential areas to improve haemophilia care.
methodsA cross-sectional, descriptive study that retrospectively reviewed clinical and laboratory records of PWH managed at DGMAH haemophilia treatment centre from 01 January 2003 to 31 December 2017.
resultsForty-four males were identified, with the majority being adults (~61%). Haemophilia A patients (~82%) outnumbered those with haemophilia B (~18%). Spontaneous mucocutaneous bleeding was the most frequent presenting feature followed by haemarthrosis. Disease-related complications included joint complications and life-threatening bleeds. There was a delay in diagnosis in 11% PWH. Management included episodic plasma-derived factor replacement and bypassing agents for patients with inhibitors. Only 13% of PWH were on home therapy. Prevalence of inhibitor development was 18%. There was a paucity of recorded data regarding prophylaxis, genetic counselling, psychological and physiotherapy support.
conclusionThe majority of PWH were adults, and haemophilia A was more prevalent than haemophilia B. A delay in haemophilia diagnosis could be addressed by increasing the awareness of haemophilia in health facilities. Expanding home therapy and introducing prophylaxis will likely improve the quality of life in PWH. Study outputs have included compilation of diagnostic and management algorithms to optimise haemophilia care at DGMAH.
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