THE PREVALENCE OF HEMOPHILIC ARTHROPATHIES IN CHILDREN WITH HEMOPHILIA IN THE VORONEZH REGION DEPENDING ON VARIOUS FACTORS
Relevance. Currently, hemophilia, leading to the formation of arthropathy, is an urgent problem in the practice of a pediatric hematologist. According to modern concepts, hemophilia is an X-linked hereditary disorder of plasma hemostasis resulting from a deficiency or absence of blood coagulation factor VIII (hemophilia A) or IX (hemophilia B). The disease primarily affects boys. The prevalence of hemophilia A is 1 case per 10,000 people; hemophilia B - 1 per 50,000 people. One of the main clinical manifestations of hemophilia is hemarthrosis, leading to the occurrence of arthropathies. Replacement therapy with factors VIII and IX is carried out in order to prevent bleeding. But despite the constant administration of clotting factors, some patients with hemophilia develop joint damage of varying severity. Hemophilic arthropathy that occurs in children with severe manifestations of the disease often leads to disability. Such studies will help to choose the most effective treatment regimen for patients with a high risk of developing arthropathy. Objective. To study the prevalence of arthropathies among children in the Voronezh region with severe and moderate forms of hemophilia receiving prophylactic administration of coagulation factor VIII or IX, and to compare the frequency of joint damage in children with hemophilia depending on various factors. Tasks To identify the frequency of development of arthropathy in children of the Voronezh region with hemophilia, including depending on age; To determine the frequency and severity of the development of hemophilic arthropathy in children under various regimens (multiplicity) of the drug for substitution therapy; Determine the frequency and severity of the development of hemophilic arthropathy with various types of input factor (plasma, recombinant); Conduct a comparative analysis of the frequency and severity of arthropathies in patients with hemophilia, depending on the above factors. Materials and methods. The outpatient records of 32 patients with moderate and severe hemophilia observed in the oncohematological center of the VODKB No. 1 were analyzed. Hemophilia was diagnosed on the basis of clinical data, anamnesis data, and data from a study of the level of factor VIII and IX in the blood serum. The severity of hemophilia was assessed according to the WHO classification of hemophilia according to severity: severe - with the level of the missing factor less than 1%, moderate - with its level from 1 to 5%. The presence and number of affected joints were assessed according to medical records. Results. All children were divided into two age groups: from 0 to 9 years old (11 patients) and over 9 years old (10-18 years old) - 21 patients. The boundary age criterion of 9 years was chosen according to WHO recommendations, due to the onset of puberty at this age. Hemophilia A was diagnosed in 27 patients (84.4%), hemophilia B - in 5 patients (15.6%). All patients were male. A severe form of the disease occurred in 11 patients (34.4%), moderate - in 16 patients (50.0%), the remaining 5 people (15.6%) had a mild form of the disease. All patients received replacement therapy with the missing clotting factor as a prophylaxis: either plasma factor was used at a dose of 30-40 U/kg (9 patients, 28.1%) or recombinant factor at a dose of 40-50 U/kg (23 patients, 71 ,9 % ). Discussion. The overall incidence of arthropathy in children with hemophilia living in the Voronezh region was 68.75%. In children under 9 years of age, this frequency was slightly lower (63.6%) compared with the group of older patients (71.4%). This may be due to the longer duration of the disease. The frequency of administration of the drug for replacement therapy (2 or 3 times a week) did not significantly affect the incidence of hemophilic arthropathy. In children receiving replacement therapy 2 times a week, joint damage was detected in 60% of cases. In children receiving replacement therapy 3 times a week, joint damage was detected in 100% of cases. The most significant influence on the incidence of hemophilic arthropathies was exerted by the type of factor administered: those who received the plasma factor had arthropathy in 70% of cases, and those who received the recombinant factor had arthropathy in 60%. Conclusion. Thus, arthropathies are the leading complication in children suffering from hemophilia, a hereditary disorder of plasma hemostasis. The decisive factor in the aggravation of this disease is age - every year the number of affected joints increases. The frequency of drug administration increased depending on the number of affected joints: arthropathy of 3 or more joints was accompanied by replacement therapy 3 times a week, in contrast to the defeat of one and 2 joints - 2 times a week. Key words: hemarthrosis, arthropathy, hemophilia, recombinant factor, plasma factor.