Abstract
To improve the results of treatment of severe hemophilia in children, the efficiency and safety of the use of venous catheter ports in patients of this group has been analyzed. The problem of optimizing vascular access in hemophilia, especially young patients, remains relevant, despite the emergence of drugs with a subcutaneous route of administration. In global clinical practice, implantable permanent venous access devices (IVAD) have been successfully used for a long time to initiate preventive therapy or to conduct immune tolerance therapy when detecting inhibitor to coagulation factor VIII as early as possible. In Russia, this experience is still local and not reflected in the literature. Materials and methods of research: a single-center retrospective uncontrolled non-randomized study was performed. In a total of 19 patients (100% boys) between the age of 1 and 8 years (median 2 years [1; 6]) have been installed 21 IVADs. The observation period ranged from 200 to 1,897 days, with a total number of 17,484 catheter days in the group. Results: a statistically significant decrease in the number of hemorrhages requiring treatment per year (p<0,001) has been observed in the general group of patients following the implantation of IVAD and the optimum treatment. The incidence of infectious complications was 0,22 per 1000 catheter days with no other complications recorded. Conclusion: the results of the presented case report demonstrate that the use of a port-type IVAD to ensure adequate vascular access in young children with severe hemophilia with a high degree of safety significantly increases the effectiveness of substitution/hemostatic therapy.
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