Patterns of Orthopaedic Complications of Haemophilia at Khartoum Haemophilia Clinic

Abstract

Background: Haemophilia is a common hereditary bleeding disorder caused by deficiency in clotting factor VIII (Type A) factor IX (Type B) with A:B 5:1. Severity of the disease depends on the level of the circulating factor. Bleeding tendency is the presenting feature and musculoskeletal
involvement is a common presenting feature.
Objectives: To study the demographic characteristics, clinical and radiological patterns of musculoskeletal disorders associated with haemophilia in patients presented to Khartoum Haemophilia Clinic (KHC).
Patients and Methods: Demographic characteristics, patterns of clinical and radiological features of 78 patients with haemophilia A and B who presented to KHC, between March 2004 and June 2005 were analyzed.
Results: There were 78 patients; all were males, their ages ranging between 1.5 and 50 years. 80% of them were either of preschool or school age groups. Haemophilia A: B was 4.5:1. Over 80% had articular involvement and the knee joint was involved in more than 50% of cases.
Radiological findings were less severe in patients with haemophilia B, and were more severe in patients older than 30 years of age.
Conclusion: We conclude that most of patients present with sequelae of recurrent musculoskeletal bleeds. Thus we observed that most of cases presented late with already destroyed joints. We recommend here to give treatment as prophylactic rather than on demand as it is now practiced as
inevitable destruction of joints with repeated bleeds will be the presenting feature.