Background. Analyzing modern medical literature, it can be noted that in pediatric rheumatology, insufficient attention is paid to assessing the trophological status of sick children. Purpose: to investigate the nutritional status of children with various nosological forms of rheumatic diseases (RD). Materials and methods. The nutritional status of 35 children with RD was investigated, of which 5 patients had systemic variant of juvenile idiopathic arthritis (JIA), 13 patients had articular form of JIA, 4 patients had systemic lupus erythematosus, 3 patients had mixed connective tissue disease (Sharp’s syndrome), 6 children had juvenile systemic scleroderma, 4 patients had juvenile dermatomyositis. All patients underwent a generally accepted comprehensive clinical, laboratory and instrumental examination. To assess the trophological status, the body mass index and the blood serum leptin were determined by the enzyme immunoassay and the trophological status coefficient was calculated. Results. The results of the conducted studies indicate that trophological insufficiency of varying degrees manifested in 78.5 % of children with RD in a decrease in body weight, depletion of muscle mass, adipose tissue, changes in the skin and its appendages, mucous membranes, organs of vision, oral cavity, cardiovascular system. The most pronounced trophological insufficiency was found in children with systemic JIA, with juvenile scleroderma and dermatomyositis.
Приведены данные о современных клинических проявлениях системной красной волчанки (СКВ) у детей и подростков с описанием полиморфизма отдельных симптомов поражения кожи, костно-мышечной системы и внутренних органов, представляющих трудности в ранней диагностике заболевания. Освещены вопросы классификационного анализа СКВ, акцентировано внимание на том, что классификационные критерии не всегда достаточны для ранней диагностики заболевания в детском возрасте, что в отдельных случаях приводит к «выпадению» больных с полностью сформированными синдромами и/или нетипичными формами заболевания. Диагностические критерии СКВ SLICC (2012) достаточно информативны, однако не нашли широкого внедрения в клинической практике детской ревматологии. Приведены классификационные критерии ACR/EULAR (2017). Представлены результаты динамического мониторинга 22 пациентов, которые в течение последних 10 лет лечились в детской клинике института. Показано, что у 28 % больных диагноз был верифицирован только при повторной госпитализации, у 10 % — почти через 6 лет после дебюта болезни. Приведено описание клинических случаев СКВ: у пациента с первично-хроническим течением (поражением кожи и костно-мышечной систем) и ребенка с подострым тяжелым течением болезни с поражением почек. Обсуждаются вопросы их диагностики в дебюте заболевания при применении классификационных критериев СКВ, предложенных ACR/EULAR (2017). Применение современных клинических и иммунологических критериев диагностики СКВ в большинстве случаев позволяет установить диагноз в ранние сроки заболевания и своевременно начать патогенетическую терапию.
Purpose — to assess the vitamin D supply in children and adolescents with systemic juvenile scleroderma (JS), taking into account its onset and clinical course at different periods of the child's development. Materials and methods. 14 children of 1 year 3 months —17 years old with systemic JS and 10 healthy children of the control group were examined. The concentration of 25(OH)D was determined in blood serum using commercial kits Vitamin D3 — Screeningkit, Switzerland, according to the manufacturer's instructions. Results. All patients with systemic JS showed a decrease in serum 25(OH)D levels — (24.55±9.32) ng/ml, compared to healthy children — (39.98±3.11) ng/ml. The lowest concentrations of the circulating form of vitamin D in the blood serum were in patients with limited form of systemic JS with Parry–Romberg hemiatrophy and «saber strike» — (14.07±3.38) ng/ml, as well as with the onset of generalized rapidly progressive JS in children at puberty — (16.31±4.6) ng/ml. Conclusions. Children with JS are shown to assess their vitamin D status by monitoring the serum concentration of 25(OH)D in order to decide whether to prescribe vitamin D supplements. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: children, juvenile scleroderma, vitamin D.
Background. The purpose was to determine the risk factors for reducing the provision of vitamin D (VD) in patients with juvenile idiopathic arthritis (JIA) and to develop a questionnaire for the rapid identification of children who require monitoring the concentration of 25OHD in the blood. Materials and methods. Clinical and laboratory examination of 78 children aged 2–17 years with JIA was performed. General clinical, biochemical, statistical methods and standardized criterion testing were used. Results. The most unfavorable risk factors for reducing VD provision in children with JIA have been identified, a questionnaire has been developed that can be used to identify people at high VD deficiency. Risk factors are evaluated in points. When questioning individuals with JIA, the total number of points obtained by each patient was determined. In children, who have received more than 264 points, VD insufficiency or deficiency is confirmed laboratorially. The electronic version of the questionnaire is created in the online service for remote testing with the ability to view results for each respondent separately and to identify patients at high risk of vitamin D deficiency. The questionnaire also allows separating groups of patients at risk of VD insufficiency for further monitoring of 25OHD concentrations in the blood serum, which is useful in clinical practice of children’s rheumatologist, pediatrician and family physician. Conclusions. In patients with JIA, it is necessary to determine the risk factors for reducing VD provision in the body to identify individuals who require the evaluation of its concentration in the blood with subsequent monitoring. A questionnaire has been developed that allows us to collect information on the risks of developing VD insufficiency, assess them and determine the basic cohort of patients for laboratory studies and deciding whether to prescribe vitamin D preparations to them.
Juvenile systemic sclerosis (JSS) has many clinical manifestations that differ from adults. Early diagnosis is problematic. The course of the disease and the severity of the prognosis depend on the involvement of internal organs in the process, first of all, the heart, lungs, kidneys. Cardiac pathology is a frequent and prognostically unfavorable target of the scleroderma process in adults, but it is rarely diagnosed in children. The aim of the work was to study the features of the clinical manifestations of systemic sclerosis in a child with severe heart disease. A polymorphism of the clinical symptoms of severe heart damage with the development of dilated cardiomyopathy in a one-year-old child with systemic sclerosis is presented. The features of the case are the early debut of systemic sclerosis in a child with a burdened hereditary history of autoimmune pathology (psoriasis in the father and grandmother), rapid progression of the autoimmune process, severe heart damage by the type of non-compact (dilated) cardiomyopathy, positive clinical dynamics when using pathogenetic therapy. Early detection of cardiovascular lesions using modern diagnostic methods, timely implementation of adequate therapy in a multidisciplinary team and regular cardiovascular screening can improve the prognosis, quality of life and reduce mortality.
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