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

Abstract

Background: Hemophilia is a rare inherited genetic blood disorder that needs a high index of suspicion and appropriate laboratory facilities for diagnosis. Unfortunately, it has not received the same patronage as other bleeding disorders like thalassemia and sickle cell anemia. Its management is expensive and requires lifelong treatment.

Methods: This is a hospital-based cross-sectional study done at a hemophilia treatment center in a tertiary hospital in South India to study the clinicoepidemiological profile and complications in hemophilia children admitted during the period of January 2022 to June 2023.

Results: 40 children with hemophilia were included in the study, and all of them had Hemophilia A with the majority being of severe type (62.5%). The mean age of diagnosis was 10 months. Hemarthrosis was the presenting complaint in about 50 % of children at admission while the majority had bruises and ecchymosis as clinical presentation at the time of first diagnosis (40%). 5 children tested positive for inhibitors among those tested. Intracranial hemorrhage was the most common occurring complication (17.5%). Knee (60%), elbow (47.5%) and ankle (40%) were the most common target joints.

Conclusions: There is a need for awareness of this disease among clinicians and people for appropriate diagnosis. The government should ensure the availability of factor replacement therapy and other treatment modalities even at District hospitals for easy accessibility of patients. Management goals should shift from on-demand therapy to prophylactic therapy for hemophilia children for better living standards and prevent complications like arthropathy in these patients.