Clinical Profile of Hemophilia patients of Upper Assam. A Hospital based Study

      Аннотация: All patients who were admitted in the Department of Medicine and Pediatrics with Hemophilia were included in the study. A total of 79 hemophilia patients were studied with 94.9% men and 4% women; 74.7% were below the age of 20 years and 78.5% were known hemophilia patients, whereas 21.5% were newly diagnosed; 64.6% patients had a definite family history; 79.7% were factor VIII deficient and 20.3% were factor IX deficient; 47 (74.6%) patients had severe factor VIII deficiency, 12 (19%) patients had moderate factor VIII deficiency and 4 (6.4%) patients had mild factor VIII deficiency; 11 (68.7%) patients had severe factor IX deficiency. Out of the 57 patients who presented with joint bleed 38 (67%) had knee joint involvement, 16 (28%) had ankle joint involvement, 13 (22.8%) had wrist involvement, 8 (14%) had elbow involvement and 3 (5.3%) had shoulder joint bleed. Out of the 19 cases of intramuscular bleed, 13 (68.4%) patients had psoas bleed; 43 (75.4%) patients had multiple joint involvement; 7(12.3%) patients both joint bleed and intramuscular bleed; 68.4% patients had joint deformity.Most of our patients would benefit from prophylactic treatment with regular factor replacement.

Ключевые слова: epidemiology, Hemophilia A and B, Northeast India, Factor VIII and IX deficiency