Clinical outcome and incidence of inhibitor development in severe hemophilia patients receiving low-dose prophylaxis: a 3-year follow-up study in Senegal, West Africa

Touré, Sokhna Aïssatou; Seck, Moussa; Sy, Diariatou; Senghor, Alioune Badara; Faye, Blaise Felix; Diop, S

doi:10.1016/j.htct.2022.04.004

Cited by 3 publications

(5 citation statements)

References 25 publications

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“…This mutation is reported in 36% [9], 38% [7] and 39.5% of severe PwH A [16] in South Africa, Senegal and Côte d'Ivoire respectively. However, in studies from Northern Africa such as Algeria and Morocco, intron 22 inversion appears to be more frequent, with frequencies of 89% [23] and 42.8% [10] respectively. Inversion of intron 1, on the other hand, is rare, with only one case reported in Algeria [17].…”

Section: F8 Gene Mutations Profile In Hemophilia a In Africamentioning

confidence: 87%

“…Overall, we identified 17 references through the electronic and manual searches that met the inclusion criteria and were considered for this systematic review. Nine of these references, studied the epidemiology of inhibitors in hemophilia A encountered in different African countries [9][10][11][12][13][14][15][16][17]. Four articles studied the factor VIII gene abnormalities involved in hemophilia A in Africa, and four others reported the FVIII gene variants associated with inhibitor development in hemophilia A [9,[18][19][20].…”

Section: Study Characteristicsmentioning

confidence: 99%

“…Four articles studied the factor VIII gene abnormalities involved in hemophilia A in Africa, and four others reported the FVIII gene variants associated with inhibitor development in hemophilia A [9,[18][19][20]. Studies were carried out in four Northern African countries (Morocco [12], Tunisia [16], Algeria [19] and Egypt [20]), two West African countries (Senegal [9,10,17] and Côte d'Ivoire [15,18]), one Central African country (Cameroon [13]) and South Africa [11,14].…”

Section: Study Characteristicsmentioning

confidence: 99%

“…The study by Touré SA et al in Senegal determined incidence of inhibitors in severe PwH A treated with low-dose factor VIII concentrates for prophylaxis. Over a 3-year period, three of the 13 severe PwH A included had developed inhibitors or an incidence of 23% [10].…”

Section: Inhibitors Incidence In Hemophilia a Patients In Africamentioning

confidence: 99%

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Systematic Review of Genetic-Related Risk Factor and Inhibitor Epidemiology in People with Severe Hemophilia a from Africa: A 2023 Update

Baglo,

Zohoun,

Azonbakin

et al. 2023

IBRR

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Background and Aims: Prevalence of factor VIII inhibitors in patients with hemophilia A varies from study to study, ranging from 15% to 30%. The important risk of inhibitor development is factor VIII mutation responsible for hemophilia A. Few studies have reported factor VIII mutations in Africa. The aim of this study was to review on FVIII gène mutations of severe hemophilia A in Africa and those associated with inhibitor development. Study Design and Methodology: A systematic review was carried out using the electronic databases Pubmed, Science Direct, Index Medicus Global and African Journals online and the key words "hemophilia A", "inhibitor", "genetic" and "Africa". Studies written in French or English on the African continent and published between 2012 and 2023 were included. Publications relating to acquired hemophilia and duplicates were excluded. In the end, 17 articles were selected. Results: The factor VIII mutations involved in severe hemophilia A in Africa are variable, consisting of intron 22 inversion, large or point deletions, nonsense and missense mutations and splicing abnormalities. Among the latter, numerous previously unrecorded mutations have been identified, and a single case of intron 1 inversion has been found in Algeria. Prevalence of factor VIII inhibitors in severe hemophilia A in Africa varies between 7,8% and 30%. Genetic abnormalities associated with inhibitors include intron 22 inversion, large deletions such as exon 1-13 deletion, nonsense mutations and c.1010-2A>G mutation. Conclusion: A better knowledge of the factor VIII mutations involved in severe hemophilia A in Africa will help improve patient management.

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Section: F8 Gene Mutations Profile In Hemophilia a In Africamentioning

confidence: 87%

Section: Study Characteristicsmentioning

confidence: 99%

Section: Study Characteristicsmentioning

confidence: 99%

Section: Inhibitors Incidence In Hemophilia a Patients In Africamentioning

confidence: 99%

See 2 more Smart Citations

Systematic Review of Genetic-Related Risk Factor and Inhibitor Epidemiology in People with Severe Hemophilia a from Africa: A 2023 Update

Baglo,

Zohoun,

Azonbakin

et al. 2023

IBRR

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“…Our patient was on low dose prophylaxis [12] but secondarily interrupted because of family constraints (moving to live with his grandparents in a very remote village with no access to electricity). This case illustrates the difficulties of following prophylaxis in a rural environment with the discontinuation of treatment and the occurrence of a CNS hemorrhage [13].…”

Section: Discussionmentioning

confidence: 99%

Untitled

2022

JHBD

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Intracranial hemorrhage (ICH) is a life-threatening complication in hemophilia. It often occurs during childhood after minimal head trauma. It poses a problem of diagnostic and therapeutic management, especially in Africa where CFCs are not always available.:We report the case of an 8-year-old boy, severe hemophiliac A, carrying sickle cell trait and trisomy 21. He was admitted to emergency 6 days after a cranioencephalic trauma following by headaches with photophobia and consciousness disorders.Cerebral CT scan showed a hematoma of the posterior cerebral fossa, a left temporal hematoma, a meningeal hemorrhage and a hydrocephalus. The patient received CFCs substitution and regular neurological follow-up.Management of ICH in hemophiliacs remains difficult in Africa because of the unavailability of treatment and the lack of knowledge of the disease. An early diagnosis and an adapted management would allow to reduce morbidity and mortality related to ICH.

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The prevalence of Hemophilia A in males in Africa: evidence from a systematic review and meta-analysis

Debela,

Bekele,

Zenbaba

2024

BMC Public Health

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Clinical outcome and incidence of inhibitor development in severe hemophilia patients receiving low-dose prophylaxis: a 3-year follow-up study in Senegal, West Africa

Cited by 3 publications

References 25 publications

Systematic Review of Genetic-Related Risk Factor and Inhibitor Epidemiology in People with Severe Hemophilia a from Africa: A 2023 Update

Systematic Review of Genetic-Related Risk Factor and Inhibitor Epidemiology in People with Severe Hemophilia a from Africa: A 2023 Update

Untitled

The prevalence of Hemophilia A in males in Africa: evidence from a systematic review and meta-analysis

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