A study of clinical characteristics and factor assays of 100 cases of Hemophilia in Bihar, India
STATUS OF HEMOPHILIA PATIENTS IN BIHAR
Background: The common bleeding disorder, hemophilia, is an X-linked genetic disorder characterized by a deficiency or absence of factor VIII (FVIII) (hemophilia A or classic hemophilia) or factor IX (FIX) (hemophilia B or Christmas disease. They are typically inherited from mother through an X chromosome with a defective gene.
Materials and method: This study was conducted in the departments of pathology of Medical Colleges and Hemophilia Superspeciality Hospital of Bihar, India, during the period of August 2017 to August 2018. Clinical parameters and factor assay result along with APTT values were systematically recorded.
Result: 81% patients had hemopilia A and 19 % patients had hemophilia B, ratio was 4.2:1. As per severity of hemophilia A, 20%, 62% and 18% were respectively mild, moderate and severe type. Severity of hemophilia was inversely proportional to level of factor assay. Hemophilia A was clinically more severe than hemophilia B for same level of factor.
Discussion and Conclusion: 54% patients presented with symptoms in the age group of 0-10 years and 1% in 55-65 years age group in this study. Mildly severe hemophilia presents in older age group.
Recurrent joint swelling was the predominant clinical finding followed by wound bleeding in this study. Hemophilia is major health problem in Bihar, India that has got huge financial and social significance Provision of proper treatment and physiotherapy is needed at hemophilia treatment centres to prevent disability and disfigurement.
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