Abstract

Hemophilia A is a hereditary coagulopathy caused by the deficiency of the coagulation factor VIII, whose main complication consists in disabling arthropathy. The most often affected joint is the one of the knee, due to which this article aims at presenting, on one hand, the role of continuous substitutive prophylactic treatment in preventing the onset of this complication and, on the other hand, the current view of orthopedic surgery in managing the above-mentioned complication. The continuous prophylactic treatment represents the best therapeutic conduct in preventing the onset of hemophilic arthropathy, yet this aspect is limited by two important factors: inappropriate medical support, dependence on the social and economic level of every country and inappropriate adherence of the patient to this thorough treatment, which represents a challenge for a life with no bleeding. Under the circumstances imposed by an insufficient substitutive treatment or by a deficient adherence of the patient to this, recurrent hemarthrosis shall lead to cartilage destruction and synovial hypertrophy (synovitis), which will impose, in time, total endoprosthesis in order to re-establish the motor function and to improve the life quality of the hemophilic patient. The surgery of the hemophilic patient is associated with an increased risk of hemorrhage and infection and it is practiced only with substitutive hematologic support. The key to the best results is the existence of a multidisciplinary experienced team, including an orthopedist, hematologist, physical therapist.

Highlights

  • OANA VIOLA BADULESCU1, MANUELA CIOCOIU1*, NINA FILIP2*, VLAD VERINGA3 1Grigore T

  • Under the circumstances imposed by an insufficient substitutive treatment or by a deficient adherence of the patient to this, recurrent hemarthrosis shall lead to cartilage destruction and synovial hypertrophy, which will impose, in time, total endoprosthesis in order to re-establish the motor function and to improve the life quality of the hemophilic patient

  • Regular substitutive therapy with deficient coagulation factor applied from a young age represents the best therapeutic conduct in order to prevent the onset of synovitis and hemophilic arthropathy

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Summary

Introduction

OANA VIOLA BADULESCU1, MANUELA CIOCOIU1*, NINA FILIP2*, VLAD VERINGA3 1Grigore T. Each hemorrhage articular episode brings about a disorder which predisposes to relapse: the anatomical structures are weakened, muscles are supposed to atrophy and suffer from fibrosis (articular mechanics becomes deficient), the synovial is supposed to hypertrophy and hypervascularize, becoming less hemorrhagic (vicious circle) This marks the beginning of a chronical condition; hemophilic arthropathy, which evolves slowly throughout life and generates severe sequelae (ankylosis). Valentino et al [10] showed, in an experimental pattern, that the hemorrhage produced by a controlled trauma leads to joint swelling, synovitis and hemophilic arthropathy To prevent these complications, regular substitutive therapy with deficient coagulation factor applied from a young age (primary prophylaxis) represents the best therapeutic conduct in order to prevent the onset of synovitis and hemophilic arthropathy. Despite primary prophylaxis, some patients present intra-articular bleeding, due to an insufficient dose of coagulation factor or a deficient adherence to the treatment, while others might show sub-clinically manifested hemarthroses

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