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Haemophilia.

E P Gétaz, W G Staples, A Lurie

    South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde
    |October 1, 1977
    PubMed
    Summary

    This review covers hemophilia (a bleeding disorder), discussing its clinical and genetic aspects. It details management challenges like factor replacement and psychological support, highlighting the South African Haemophilia Foundation's role.

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    Area of Science:

    • Hematology
    • Genetics
    • Clinical Medicine

    Background:

    • Hemophilia is a genetic bleeding disorder with significant clinical manifestations.
    • Understanding its historical context, pathogenesis, and genetics is crucial for effective management.

    Purpose of the Study:

    • To provide a comprehensive review of hemophilia.
    • To discuss the multifaceted challenges in managing patients with hemophilia.
    • To highlight the supportive role of organizations like the South African Haemophilia Foundation.

    Main Methods:

    • Literature review of clinical and haematological features.
    • Discussion of historical, pathogenetic, and genetic aspects.
    • Description of management strategies and associated problems.

    Main Results:

    • Detailed overview of clinical and haematological presentations.
    • Exploration of historical evolution, pathogenesis, and genetic underpinnings.
    • Comprehensive description of management issues including factor replacement, home therapy, physiotherapy, surgical interventions, and inhibitor presence.

    Conclusions:

    • Effective hemophilia management requires addressing clinical, genetic, and logistical challenges.
    • Psychosocial support is integral to patient care.
    • The South African Haemophilia Foundation provides vital assistance to individuals with hemophilia.

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