Hemophilia in children: a clinico-epidemiological profile and review
Abstract
Introduction: Objective: To describe the clinical and epidemiological profile of hemophilia patients in the pediatric age group at a tertiary care center in Western Uttar Pradesh.
Materials and Methods: This was an observational cross-sectional study that was conducted at the Department of Pediatrics, S.N. Medical College, Agra. Pediatric patients (0-18 years) with hemophilia admitted in the Pediatrics emergency ward for Anti- Hemophilic factor transfusion or having a history of such type of transfusion during the year 2018-19 were consecutively enrolled in the present study. A detailed clinical history was taken from the parent or guardian accompanying the case.
Results: Out of the total 93 pediatric hemophilia patients, the majority 61% (57/93) belonged to 11 to 18 years of age group. 2.1% (2/93) were< 1 year of age. 17.2 % (16/93) were in 1-5 years of age group. 19.35% were in 6-10 years of age group. Out of the total, 98.9% (92/93) were males while only 1.07% (1/93) were female. Type A hemophilia was observed in 87.09% (81/93) patients while Type B hemophilia was present in only 12.9% (12/93) patients. Out of 81 Type-A hemophilia patients, only one had developed Inhibitor (1.07%). None among Type B hemophilia patients had Inhibitors. Out of total cases, only one patient (1.07%) had von Willebrand Deficiency.91%(85/93) patients presented with joint bleed. The knee joint was the most commonly affected joint, as observed in 64.7% (55/85) patients.
Conclusion: A better insight into the disease prevalence is pivotal for early diagnosis and to provide quality care to all hemophilia patients. The development of an inhibitor is a major concern. Physiotherapy and RICE(Rest, Ice, Compression and elevation) should be emphasized for the better quality of life and preventing joint hemarthrosis.
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