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.

Clinical profile of hemophilia children admitted in a tertiary care hospital in South India: a cross sectional study

“…No significance was seen between the severity of hemophilia and the occurrence of intracranial bleeds in the children. [3][4][5] All these studies show a higher incidence of Hemophilia A than Hemophilia B, as supported by Franchini and Mannucci. 6 As per studies, Hemophilia A accounts for about 84% of all hemophilia patients in India.…”

“…Children with severe hemophilia had comparatively more spontaneous onset of bleeding than others (9), and few had more than 10 bleeding episodes per year (3). No significance was seen between age at the time of diagnosis and severity of hemophilia.…”