Clinical Profile of Hemophilia in Children in a Tertiary Care Hospital in North India.

Hemophilia is congenital X-linked disorder of the coagulation system whereby deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) predisposes the affected male to a lifelong risk of bleeds. Early and adequate support with factor replacement will prevent death from fatal bleeds. However, awareness levels among students and general practitioners continued to be very low resulting in delayed and inappropriate management. The present study designed to focus on the presentation, genetic aspects and management of hemophiliac children. Clinical profile of 119 diagnosed cases of hemophilia up to 18 years of age was analyzed. Out of a total 119 cases enrolled mean age of the patients was 9.5 years with an age range of 6 months to 18 years. 97(81%) cases were hemophilia A and 22 (18.4%) cases were hemophilia B. Only 48(40.0%) cases had family history of bleeding with 71 (60%) had no history of bleeding in family. Among the hemophilia A 50.4% (52.5%) cases had mild, 45.2 (47.5%) cases had moderate disease and among the hemophilia B, 9.5 (40%) cases had mild, 11.5 (50%) cases had moderate and 2.3 (10%) cases had severe disease. Hemarthrosis of knee joint was the major presentation followed by ankle, elbow, shoulder followed by circumcision and tooth extraction bleeding. 62% of the hemophiliacs in our study had initial bleeding episode before 1 year of age and by 6 years of age 94% of cases had developed symptoms of bleeding evidence. No patient had history of bleeding during neonatal period. It is concluded that hemarthrosis found to be the leading cause of presentation; bruises and hematomas either spontaneous or traumatic were the chief complaints at the presentation of these children. [ABSTRACT FROM AUTHOR]

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