Tahira Zafar, Nadeem Ikram, Samina Amanat, Asif Zafar, Khalid Hassan.
Clinico - hematological spectrum of hemophilia
J Rawal Med Coll Dec 2006;10(2):54-60.
Haemophilia Patients Welfare Society, Rawalpindi

Background: To assess status of the commonest inherited bleeding disorder Haemophilia, and to critically appraise services available for its patients. Methods: Patients presenting to Haemophilia Patients Welfare Society (HPWS) were included in the study. Demographic data, lab findings, estimation of disease severity, burden of disability, bleeding patterns and treatment given to patients of Haemophilia were recorded. Results: Out of 117 cases 88.88% cases were of Haemophilia A and 11.11% cases were of Haemophilia B. Family history of haemophilia was found in 56.41% cases. On clinical stratification severe, moderate and mild haemophilia was seen in 55.55%, 18.8% and 25.64% cases, respectively. Permanent disability and arthropathies were found in 31.62% and 75.21%. cases. Out of total recorded episodes 74.35% occurred spontaneously. Knee joint was the most commonly involved joint (47.83% episodes). Sixty two percent episodes were treated with factor concentrates. Majority of episodes (72%) responded to a single infusion. Un-indicated joint aspiration and inhibitors development claimed the life of two patients. Conclusion: Establishing comprehensive haemophilia care centres, orthopaedic interventions, physiotherapy, psychological support, prenatal and perinatal counselling, productive adjustment of haemophiliacs in the society, prophylactic factor replacement and awareness of the problem on higher level are the issues which once dealt with can change the dismal view of this disease.

Category: Haematology
Keywords: Hemophilia. Clotting Disorders.

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