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.
Highlights
To describe the clinical and epidemiological profile of hemophilia patients in the pediatric age group at a tertiary care center in Western Uttar Pradesh
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
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
Summary
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. Results: Out of the total 93 pediatric hemophilia patients, the majority 61% (57/93) belonged to 11 to 18 years of age group. Out of 81 Type-A hemophilia patients, only one had developed Inhibitor (1.07%). Only one patient (1.07%) had von Willebrand Deficiency.91%(85/93) patients presented with joint bleed. Hemophilia is an X-linked recessive bleeding disorder caused by the deficiency of coagulation factors VIII, IX, or XI. Factor XI deficiency is commonly known as Hemophilia C. It is the milder variant and mostly seen in Ashkenazi Jews. Being an Xlinked recessive disorder, males are affected and females are asymptomatic or mildly affected carriers
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