Clinical profile of Hemophilia in Children in a tertiary care centre in Andhra Pradesh, India

Lakshmi Chaitanya Varma Pusapati; Dr. Srinivasa Rao Majeti

Volume : V, Issue : X, October - 2015

Clinical profile of Hemophilia in Children in a tertiary care centre in Andhra Pradesh, India

Lakshmi Chaitanya Varma Pusapati, Dr. Srinivasa Rao Majeti

Abstract :

Introduction: Hemophilias are a group of inherited, life–long coagulation disorders. Hemophilia A (classic hemophilia, factor VIII deficiency) and hemophilia B (Christmas disease, factor IX deficiency) are sex–linked recessive disorders, with the incidence of hemophilia A outnumbering those with hemophilia B. Some of the patients have their disorder caused by mutation. Hemophilias are present in every nook and corner of the world and have a varied presentation. There is a high morbidity and mortality associated with it and knowing their presentation helps in better management practices. Objectives: To observe the clinical presentation of hemophilia in children. Methodology: This observational study was carried out in the Department of Pediatrics, Alluri Sitarama Raju Academy of Medical Sciences for a period of one year from 1st Feuary 2014 to 31st January 2015. Clinical profile of 66 diagnosed cases of hemophilia <15 years of age was analyzed. Result: All the cases under the study were male with an age range of 3 months to 15 years. Hemophilia A was seen in 51(77%) cases and 15(23%) cases were identified as hemophilia B. Only 49% cases had family history of bleeding. Among hemophilia A, 49% were mild, 39% were moderate and 12% with severe hemophilia. 53.34% of hemophilia B presented with mild disease, 33.33% with moderate form of the disease and 13.33% with severe disease. Hemarthrosis (68%) was the most common problem with which the patients presented. Gum bleeding was the second most common presentation followed by prolonged bleeding after tooth extraction, uises and Ecchymosis. 49% of the patients had their first bleeding symptoms between ages of 1–5 years and 85 %cases showed bleeding manifestation before the age of 5. Only 15% cases had presented after 5 years of age. Bruises and hematoma were the most common initial manifestation followed by joint bleeding, cut injury of lips and chin, scalp and facial hematoma. Conclusion: Bruises and hematoma were the main presentation features of the children in the study. Children with hemarthrosis have a rather morbid childhood. Cases as high as 36% of them presented before their first birthday which makes mandatory detection in infants.