Abstract

Hemophilia A and B are clinically indistinguishable and are heterogeneous disorders. The severity of bleeding symptoms correlates with the coagulant activity of the deficient factor. Joint bleeding initially leads to independent adverse changes in both the synovial tissue and the articular cartilage. The aim of the present work was to evaluate hemophilic joints clinically, radiologically, and functionally in patients with hemophilic arthropathy. The study was carried out on 30 boys suffering from hemophilic arthropathy; the mean age was 10.6 ± 2.95 years. All patients were subjected to thorough history taking and local physical examination of the ‘target joint’. Functional Independence Score in Hemophilia (FISH) and the Pettersson scoring system were assessed for all patients. The age at first hemarthrosis decreased with the severity of hemophilia, whereas the number of bleeds/year and the number of joints affected increased with the severity, and the results were statistically significant. A statistically significant positive correlation was found between the Pettersson score and both the age of the patients and the number of bleeds/year. However, a negative correlation was found with factor activity level. In contrast, the FISH score had a significant positive correlation with factor activity level. A significant decrease in the functional ability was demonstrated on the basis of the severity of hemophilia. Both the FISH and Pettersson scoring systems are of great importance in assessing patients with hemophilic arthropathy.

Highlights

  • Hemophilia A and B are clinically indistinguishable and are heterogeneous disorders

  • Joint bleeding initially leads to independent adverse changes in both the synovial tissue and the articular cartilage

  • The age at first hemarthrosis decreased with the increased severity of hemophilia, whereas the number of bleeds/year and the number of joints affected increased with the increased severity and the results were statistically significant (P = 0.001, P < 0.001, and P = 0.048, respectively) (Table 1)

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Summary

Introduction

Hemophilia A and B are clinically indistinguishable and are heterogeneous disorders. The severity of bleeding symptoms correlates with the coagulant activity of the deficient factor. Hemophilia A and B are clinically indistinguishable and are heterogeneous disorders [1,2]. Their clinical manifestations are identical, with an increased tendency for musculoskeletal, soft tissue, and mucocutaneous bleeding. Joint bleeding initially leads to independent adverse changes in both the synovial tissue and the articular cartilage. Both synovial inflammatory changes and cartilage damage affect each other [6]

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