The relevance of this problem research is conditioned on the intellectually trained teaching staff shortage and the complexity of organizing pre-professional teacher training in high school. The purpose of this article is to develop and test the program named "Intellect" for students of pedagogical specialized classes. The content of the program has an intellectual, emotional-value, communicative holistic impact on students: The leading research method for this problem was the questionnaire survey, that was conducted in 114 high school students and 13 teachers, which allowed to identify positive dynamics in high school students attitude to modern education and pedagogical activity. The article proves that an expanded psychological and pedagogical intelligence-based education can be the constructing successful life trajectories foundation for high school students The presented in the article research results indicate that the «Intellect» program implementation give rise to intellectual (cognitive) activity of high school students; pedagogical education professional preferences cultivation; activation of essential in the pedagogical field personal qualities of high school students; students self-development. Practical relevance of the research results is the ability to use the presented program to organize pedagogical oriented specialized classes.

Background. Polyarteritis nodosa is an acute, subacute or chronic immune complex disease associated with peripheral and visceral artery involvement, predominantly of middle and small sizes, development of destructive-proliferative arteritis and subsequent peripheral and visceral ischaemia.Cases description. The present paper describes two clinical cases of polyarteritis nodosa in patient R., aged 12, and patient A., aged 9, and demonstrates the difficulties of diagnosing the disease in its early stages. Patient R., aged 12, was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital with complaints of red, patchy, dense rash on the palms and plantar surface of the feet. The child has been ill since September 2017, and after a history of tonsillitis suffered a fever of 37 °C, pain in the right heel area, nodular thickening on the feet, livedo reticularis, swelling of both hands. The disease had a recurrent course. The boy was treated with prednisolone, mycophenolate mofetil, hydroxychloroquine and three courses of rituximab (April 2018, January 2019, September 2020). Repeated courses of human normal immunoglobulin and alprostadil therapy were carried out. The treatment showed positive dynamics, fever was eliminated, general well-being improved, and acute inflammatory markers in blood became normal. The skin retained minimal manifestation of livedo, nodularities on the feet did not progress in dynamics. Patient A. was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital in April 2022 with complaints of weakness, myalgia of the lower extremities and necrosis foci in the left lumbar region. The medical history indicates that in March 2022, the boy, being in good health before, developed a bluish, painful rash on his lower legs after a workout. Skin changes and soreness resolved on their own without treatment. After examination, a diagnosis was made as follows: juvenile polyarteritis nodosa, activity score — 3.Conclusion. The diagnosis of polyarteritis nodosa can be often problematic due to the very character of the disease featured by absence of specific symptoms, by polymorphism of clinical manifestations, and by lack of clear diagnostic and laboratory markers.

Aim. The research was conducted to study the structure (variants, sexual and age characteristics) and manifestations of articular syndrome in children with juvenile idiopathic arthritis in Krasnodar.Materials and methods. There was conducted a retrospective analysis of 89 case histories in patients aged 2-15 diagnosed with juvenile idiopathic arthritis for the first time.Results. As a result of the study, the following variants of juvenile idiopathic arthritis were diagnosed: oligoarticular – in 62 patients (69,6%), polyarticular – in 24 patients (27%) and systemic – in 3 children (3,4%). Group 1 consisted of 62 patients with oligoarticular variant, and group 2 consisted of 24 patients with polyarticular variant. In the sexual aspect, there were 1,5 times more girls than boys among all the studied children. There are also a higher number of the females within the groups. The average age of the onset of the disease in all patients with juvenile idiopathic arthritis was 8,9±0,4 years. In group 1, the average age was 9,6±0,5 years, in group 2 – 7,4±0,4 years. In children with oligoarticular variant of the disease, knee and ankle joints were more often involved in the process. In group 2, the pathological process involved both large joints and small joints of the hands and feet. Leukocytosis, accelerated erythrocyte sedimentation rate and positive results of immunological studies were more often detected among the children of the second group. On the radiographs, cartilage and bone changes were found in 9% of patients.Conclusion. Among the patients with juvenile rheumatoid arthritis, the residents of Krasnodar, the oligoarticular variant dominates (69,7%), the polyarticular RF-negative variant is in second place (25,8%), the systemic variant is in third place (3,4%), and the polyarticular RF-positive variant is in last place (1,1%). In the sexual aspect, there were 1,5 times more girls than boys among all the patients. The onset of the joint syndrome mainly occurs in early school and puberty age. The articular syndrome was manifested mainly by the arthritis of the knee, ankle joints, and the polyarticular variant was also manifested by the arthritis of the wrist joints and small joints of the hands and feet. Among the patients, the signs of humoral activity and immune inflammation were more characteristic of the polyarticular variant. At the onset of disease, the changes of cartilaginous and bony tissues of the joints were identified by the radiography only in 9% of cases.

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

Background. Mucopolysaccharidosis type IVA (Morquio syndrome) is a rare genetic lysosomal storage disease. Due to rarity, the syndrome is typically diagnosed at a later stage of gross affections of musculoskeletal and central nervous systems, leading to disability and a markedly reduced quality of life. A replacement therapy is nowadays available with recombinant human N-acetylgalactosamine-6-sulfatase (elosulfase alfa) enzyme.Clinical cases description. Two siblings, 10-yo male and 8-yo female, were admitted with complaints of growth retardation, deformity of the spine, thorax and joints, impaired hearing and visual acuity, poor tolerance to exercise. In the boy’s medical history, first manifestations appeared in the first year of life and progressed gradually; the patient was being observed as spondylodysplastic. Mental development was unaffected. The diagnosis was confirmed only by age of 7 at the National Medical Research Center for Children's Health Federal State Autonomous Institution of the Ministry of Health of the Russian Federation. Genotyping revealed two SNP mutations in gene GALNS (g.88909227C>A and g.88884454G>A in heterozygous state), and enzymatic assays — a severely reduced N-acetylgalactosamin-6-sulfatase activity. A routine elosulfase alfa replacement therapy has been received since 8-year age.The younger sister had neonatal cardiomegaly; congenital carditis and cardiomyopathy not excluded. Musculoskeletal affections developed by age of 3–4 years. By age of 5 and simultaneously with brother, the same GALNS mutations and severely impaired N-acetylgalactosamine-6-sulfatase activity were detected. A replacement therapy has been routinely received since 6-year age. The therapy triggered positive dynamics of restoring activity and muscle strength in both children, as well as significantly abating the musculoskeletal affection progress.Conclusion. The clinical cases of Morquio syndrome presented demonstrate its long-term and complex diagnosis. A replacement therapy is nowadays available, which warrants an earliest disease detection to halt progression and improve the patient’s life quality and expectancy.