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We describe the diagnosis of and treatment for brain compression on background of hemostasis disorders in 6 out of 37 patients with hemorrhagic strokes treated in the Ivano-Matreninskaya Children’s Clinical Hospital in 2017–2018. Laboratory examination revealed factor VIII deficiency (hemophilia A) in 3 patients, factor XIII deficiency in one patient; 2 children were carriers of thrombophilia genetic polymorphisms. The age of study participants varied between 15 days and 2 years; all of them were males. They have undergone neurological examination, laboratory testing (hemostasis), neurosonography, multislice computed tomography, and magnetic resonance imaging. Conservative therapy with quarantined fresh frozen plasma (15–25 mL/kg) was used to correct coagulopathy and stop bleeding. Two patients had surgeries: decompressive craniectomy and drainage and endoscopic removal of hematoma. Children that were operated on received transfusion of fresh frozen plasma and red blood cells. Both of them had regression of neurological symptoms and restoration of consciousness in the postoperative period. None of the patients died. In this article, we discuss diagnostics and strategy of conservative and surgical treatment for children with brain compression and coagulopathies.

Hemophilia B is a recessive X-linked bleeding disorder characterized by deficiency of the coagulation factor IX (FIX). In hemophilia B patients the severity of the bleeding phenotype is related to the degree of the FIX defect. Hemophilia B treatment has improved greatly in the last 20 years with the introduction first of plasma-derived and then of recombinant FIX concentrates. Replacement therapy may be administered through on-demand or prophylaxis regimens, but the latter treatment modality has been shown to be superior in prevention of hemophilic arthropathy and in improvement of patients' quality of life. The purpose of this narrative review is to summarize the current knowledge on treatment strategies for hemophilia B, focusing on recombinant FIX products either clinically used or in development. There is only one rFIX product that is licensed to treat hemophilia B patients; from the analysis of the literature data presented in this review, the authors conclude that this rFIX product has demonstrated an excellent safety profile and excellent clinical efficacy for halting and preventing bleeds in hemophilia B patients. While prophylaxis has emerged as the best therapeutic strategy for such patients because of its ability to prevent hemophilic arthropathy and to improve patients' quality of life, the pharmacokinetically tailored dosing of rFIX is another key point when planning hemophilia B treatment, as it allows optimization of the factor concentrate usage. Further clinical studies are needed to better assess the safety and efficacy (ie, the incidence of adverse reactions and inhibitor development) of newer rFIX products. [ABSTRACT FROM AUTHOR]