Background. Combined injuries involving brain damage represent the most severe and life-threatening conditions in hemophilia patients. These injuries are characterised by specific situational and behavioural circumstances indicating the presence of victim behaviour in such patients.Aim. To analyse the influence of victim behaviour in hemophilia patients on the formation of combined neurosurgical trauma and the choice of neurosurgical and traumatological treatment approaches.Materials and methods. Twenty five patients (20 patients with hemophilia A and 5 patients with hemophilia B) were included in the study. The patients suffered the following injuries: craniocerebral injuries — 68 (100.0 %); bone fractures — 18 (26.6 %); hematomas of the soft tissues of the face, upper and lower extremities, as well as bruised, lacerated wounds — 50 (73.4 %).Results. The following types of victim behaviour were identified in the hemophilia patients: paranoid — 7 (28.0 %), dependent — 8 (32.0 %), dissociative — 6 (24.0 %) and antisocial — 4 (16.0 %). The patients underwent hemostatic therapy with coagulation factor VIII or IX concentrates and surgical (neurosurgical and/or traumatological) treatment of the injuries associated with victim behaviour. In 51 (75.0 %) cases there was delayed medical care, which was the reason for the complicated course of the post-traumatic period. It was revealed that the best treatment results in patients with severe injuries, including craniocerebral traumas, were achieved in cases where medical assistance was provided in the first three hours after injury. As a result of the treatment, the majority of the patients demonstrated regression of the clinical manifestations of the injury.Conclusions. The proposed tactics for the diagnosis and neurosurgical/traumatological treatment of hemophilia patients with signs of victim behaviour, in whom combined brain injuries are detected, includes a comprehensive assessment of medical history data, clinical and laboratory examination, as well as determination of diagnostic criteria for the choice of patient-specific surgical techniques. It is proposed to use the number of patients’ return visits related to their victim behaviour as a quantitative assessment of the degree of victimization.Conflict of interest: the authors declare no conflict of interest.
Introduction. Hemophilia is an X-linked hereditary blood clotting disorder caused by insufficiency of blood clotting factor VIII or IX that affects mainly men. In extremely rare cases, the disease can be observed in women, which is most often associated with asymmetric inactivation of the X chromosome. The severity of hemophilia in women does not differ from that in men. Aim – to present a clinical observation of surgical treatment of stage 4 hemophilic arthropathy in a woman with severe hemophilia A. Main findings. Female patient T., 39 years old, was admitted to the National Medical Research Center for Hematology with a preliminary diagnosis: hereditary deficiency of factor VIII. She had an extension of the APTT to 68.8 sec, a high level of Willebrand factor activity — 222 %, and the concentration of Willebrand factor antigen of 178.1 mg/l, and a decrease in the level of factor VIII to 1.6 %. According to molecular genetic analysis, intron 22 inversion associated with severe hemophilia A was detected in the F8 gene. Throughout the patient’s life, hemarthrosis of the knee, ankle and elbow joints were observed, which led to the development of severe arthropathy of varying severity. The woman underwent total knee arthroplasty and arthrolysis of the left ankle joint. The postoperative period proceeded without complications. Hemostatic replacement therapy was performed with a recombinant factor VIII.
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