European Journal of Clinical and Biomedical Sciences

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Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa

Received: 11 June 2023    Accepted: 11 July 2023    Published: 31 August 2023
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Abstract

Background: Hemophilia is a congenital, inherited bleeding disorder that prevents blood from clotting, causing abnormally long bleeding. It remains underdiagnosed in Africa and particularly in the Democratic Republic of Congo, where it remains poorly known to the population and health professionals. The present study aims to determine the clinical profile and cardiovascular risk factors in hemophiliacs at the University Clinics of Kinshasa. Methods: This is a cross-sectional study with an analytical aim, in which 155 patients (children and adults) with a haemorrhagic syndrome were included. The diagnosis of haemophilia was confirmed in thirty-six patients, all male; 22 hemophiliac patients regularly followed were matched with 24 non-hemophilic patients (control group). Results: hemophilia A represented 81% and hemophilia B 19%. The most represented age group was between 3 to 6 years (19%). Regarding the severity of the disease, half of the patients, or 50%, presented with severe hemophilia, the main symptom being hemarthrosis (30%). Of our total sample of hemophiliacs, we had recorded two cases of death (5.5%). Regarding cardiovascular risk factors, only the deterioration of myocardial function was found in one patient (4.5%). Conclusion: hemophilia is a disabling disease, which alters the quality of life of patients. Cardiovascular risk factors seem to be lower in Congolese hemophiliacs, however, ultrasound monitoring is necessary in order to prevent any pejorative evolution.

DOI 10.11648/j.ejcbs.20230903.11
Published in European Journal of Clinical and Biomedical Sciences (Volume 9, Issue 3, June 2023)
Page(s) 28-37
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Hemophilia, Cardiovascular Risk Factors, Clinical Profile, University Clinics of Kinshasa

References
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[3] Alperstein W, Corrales F, Tamariz L, Palacio G and Davis J. Prevalence of Hypertension (HTN) and cardiovascular risk factors in a hospitalized paediatric Haemophilia population, J Pediatr Hematol. Oncol. 40 (2018) 196–199.
[4] Franchini M and Mannucci PM. Co-morbidities and quality of life in elderly persons with haemophilia. Br J Haematol 2010; 148: 522-33.
[5] Fransen van de Putte DE, Fischer K and Pulles AE. Non-fatal cardiovascular disease, malignancies, and other co-morbidity in adult haemophilia patients. Thromb Res 2012; 130: 157–62.
[6] Zeynep C, Pelin K, and Birsen U. Myocardial functions, blood pressure changes, and arterial stiffness in children with severe haemophilia A. Original article journal homepage: www.elsevier.com/locate/thromresl.
[7] Pocoski J., A. Ma, CM Kessler, S. Boklage and TJ Humphries. Cardiovascular comorbidities are increased in US patients with haemophilia A: a retrospective database analysis, Haemophilia 20 (2014) 472–478, https://doi.org/10.1111/hae. 12339.
[8] Ragni MV and Moore CG. Atherosclerotic heart disease: prevalence and risk factors in hospitalized men with haemophilia A. Haemophilia 2011; 17: 867–71.
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[15] Chambost A and Meunier S. Challenges of early pediatric management of severe hemophilia. Archives of Pediatrics. 2006; 13: 1423-30.
[16] Sharathkumar A, Soucie J, Trawinski B, Greist A, Shapiro A et al. Prevalence and risk factors of cardiovascular disease (CVD) events among patients with haemophilia: experience of a single haemophilia treatment center in the United States (US). Haemophilia 2011; 17: 597–604.
[17] Rosendaal FR, Smit C, Varekamp I, Bröcker-Vriends AH, Van Dijck H, Suurmeijer TP, et al. Modern haemophilia treatment: medical improvements and quality of life. J Intern Med 1990; 228: 633-640.
[18] Kulkarni R, Soucie JM and Evatt BL, Hemophilia Surveillance System Project Investigators. Prevalence and risk factors for heart illness among males with haemophilia. Am J Hematol 2005; 79: 36–42.
[19] Wong M, Macgregor D, Stuckless S, Barrett B, Kawaja M and Scully MF. Health-related quality of life in a cohort of adult patients with mild hemophilia A. J Thromb Haemost 2008; 6: 755–761.
[20] Yidiz R, Miesbach W, Alesci S, Krekeler S, and Seifried E. Comorbidities and bleeding pattern in elderly haemophilia A patients. Haemophilia 2009; 15: 894–9.
[21] Staritz P, de Moerloose P, Schutgens R and Dolan G. Applicability of the European Society of Cardiology guidelines on management of acute coronary syndromes to people with haemophilia- an assessment by the ADVANCE Working Group. Haemophilia 2013; 833–40.
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  • APA Style

    Gueth Kundabi, Gibency Mfulani, Karim Assani, Nina Domo, Aleine Budiongo, et al. (2023). Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa. European Journal of Clinical and Biomedical Sciences, 9(3), 28-37. https://doi.org/10.11648/j.ejcbs.20230903.11

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    ACS Style

    Gueth Kundabi; Gibency Mfulani; Karim Assani; Nina Domo; Aleine Budiongo, et al. Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa. Eur. J. Clin. Biomed. Sci. 2023, 9(3), 28-37. doi: 10.11648/j.ejcbs.20230903.11

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    AMA Style

    Gueth Kundabi, Gibency Mfulani, Karim Assani, Nina Domo, Aleine Budiongo, et al. Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa. Eur J Clin Biomed Sci. 2023;9(3):28-37. doi: 10.11648/j.ejcbs.20230903.11

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  • @article{10.11648/j.ejcbs.20230903.11,
      author = {Gueth Kundabi and Gibency Mfulani and Karim Assani and Nina Domo and Aleine Budiongo and Jonathan Kukila and Eric Mukenge and John Senga and Bodi Mabiala and René Ngiyulu and Jean-Lambert Gini},
      title = {Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa},
      journal = {European Journal of Clinical and Biomedical Sciences},
      volume = {9},
      number = {3},
      pages = {28-37},
      doi = {10.11648/j.ejcbs.20230903.11},
      url = {https://doi.org/10.11648/j.ejcbs.20230903.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ejcbs.20230903.11},
      abstract = {Background: Hemophilia is a congenital, inherited bleeding disorder that prevents blood from clotting, causing abnormally long bleeding. It remains underdiagnosed in Africa and particularly in the Democratic Republic of Congo, where it remains poorly known to the population and health professionals. The present study aims to determine the clinical profile and cardiovascular risk factors in hemophiliacs at the University Clinics of Kinshasa. Methods: This is a cross-sectional study with an analytical aim, in which 155 patients (children and adults) with a haemorrhagic syndrome were included. The diagnosis of haemophilia was confirmed in thirty-six patients, all male; 22 hemophiliac patients regularly followed were matched with 24 non-hemophilic patients (control group). Results: hemophilia A represented 81% and hemophilia B 19%. The most represented age group was between 3 to 6 years (19%). Regarding the severity of the disease, half of the patients, or 50%, presented with severe hemophilia, the main symptom being hemarthrosis (30%). Of our total sample of hemophiliacs, we had recorded two cases of death (5.5%). Regarding cardiovascular risk factors, only the deterioration of myocardial function was found in one patient (4.5%). Conclusion: hemophilia is a disabling disease, which alters the quality of life of patients. Cardiovascular risk factors seem to be lower in Congolese hemophiliacs, however, ultrasound monitoring is necessary in order to prevent any pejorative evolution.},
     year = {2023}
    }
    

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  • TY  - JOUR
    T1  - Clinical Profile and Cardiovascular Risk Factors Among Hemophilians Followed at University Clinics of Kinshasa
    AU  - Gueth Kundabi
    AU  - Gibency Mfulani
    AU  - Karim Assani
    AU  - Nina Domo
    AU  - Aleine Budiongo
    AU  - Jonathan Kukila
    AU  - Eric Mukenge
    AU  - John Senga
    AU  - Bodi Mabiala
    AU  - René Ngiyulu
    AU  - Jean-Lambert Gini
    Y1  - 2023/08/31
    PY  - 2023
    N1  - https://doi.org/10.11648/j.ejcbs.20230903.11
    DO  - 10.11648/j.ejcbs.20230903.11
    T2  - European Journal of Clinical and Biomedical Sciences
    JF  - European Journal of Clinical and Biomedical Sciences
    JO  - European Journal of Clinical and Biomedical Sciences
    SP  - 28
    EP  - 37
    PB  - Science Publishing Group
    SN  - 2575-5005
    UR  - https://doi.org/10.11648/j.ejcbs.20230903.11
    AB  - Background: Hemophilia is a congenital, inherited bleeding disorder that prevents blood from clotting, causing abnormally long bleeding. It remains underdiagnosed in Africa and particularly in the Democratic Republic of Congo, where it remains poorly known to the population and health professionals. The present study aims to determine the clinical profile and cardiovascular risk factors in hemophiliacs at the University Clinics of Kinshasa. Methods: This is a cross-sectional study with an analytical aim, in which 155 patients (children and adults) with a haemorrhagic syndrome were included. The diagnosis of haemophilia was confirmed in thirty-six patients, all male; 22 hemophiliac patients regularly followed were matched with 24 non-hemophilic patients (control group). Results: hemophilia A represented 81% and hemophilia B 19%. The most represented age group was between 3 to 6 years (19%). Regarding the severity of the disease, half of the patients, or 50%, presented with severe hemophilia, the main symptom being hemarthrosis (30%). Of our total sample of hemophiliacs, we had recorded two cases of death (5.5%). Regarding cardiovascular risk factors, only the deterioration of myocardial function was found in one patient (4.5%). Conclusion: hemophilia is a disabling disease, which alters the quality of life of patients. Cardiovascular risk factors seem to be lower in Congolese hemophiliacs, however, ultrasound monitoring is necessary in order to prevent any pejorative evolution.
    VL  - 9
    IS  - 3
    ER  - 

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Author Information
  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Department of Anesthesia-Resuscitation, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Department of Hematology, Department of Medical Biology, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Department of Hematology, Department of Medical Biology, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Cardiology Service, Department of Paediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Emergency Department, Department of Paediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

  • Hematology-Oncology Service, Department of Pediatrics, Kinshasa University Clinics, Faculty of Medecine, University of Kinshasa, Kinshasa, DR Congo

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