Abstract
Introduction: Worldwide Haemophilia A (HA) is the most common coagulation factor deficiency. It affects all populations and its prevalence varies among different countries, but roughly prevalence is estimated to be of 3-20 cases per 100,000 population. The classification of HA provides a guidance to possible types of bleeding and the rate of occurrence of hemorrhagic episodes. The hemophiliac patients with severe form, can be presented with spontaneous bleeding and hemorrhage after minor trauma about 1-6 times in a month, including hemarthrosis and intramuscular hemorrhage. In moderate form, the patients usually experience bleeding after mild to moderate injuries, while HA patients with mild form may not be diagnosed for years and bleed after surgery or major trauma. About 70% of children who have a positive history of HA in the family are diagnosed at birth or after the first bleeding episode.
 Material and Methods: A complete medical history of the patient was taken with reference to the name, age, sex, address, occupation, history of present illness, duration illness, education of patient and parent. Previous history of treatment, past and personal history and socio-economic status were noted. Parents were explained the purpose of the study and the procedures involved.The Hemophilia Joint Health Score (HJHS) was used to assess joint health in children included in the study.
 Results: Age of diagnosis >=12 months was observed in 28 (54.90%) of cases while in 23 (45.10%) cases age of diagnosis was < 12 months. Out of 51 cases Severe disease was observed in 45(88.23%) of cases, moderate in 4(7.84%) and mild form was observed in 2(3.95%).Out of total 51 cases 42 (82.4%) were from the nuclear family while 9(17.6%) were from the joint family. Mean HJHS was higher in patients of nuclear family as compared to joint family.
 Conclusion: HJHS shows construct validity and is more sensitive for mild arthropathy and can be used for studies in children with hemophilia. No correlation of different demographic parameters in children suffering from hemophilia A was seen.
 Keywords: HJHS, HA, Hemophilia A and B, CDC, HTCs, UDC,FA.
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