Кафедра педіатрії № 1 та неонатології
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Browsing Кафедра педіатрії № 1 та неонатології by Subject "2023а"
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Item Association of Myocardial Changes and Gene Expression of the NFATC1 and NFATC4—Calcineurin Signaling Pathway in Children with Bicuspid Aortic Valve /(2023) Kamenshchyk, Andrii; Gonchar, Margaryta; Oksenych, Valentyn; Kamyshnyi, AleksandrBackground: The role of NFATC gene expression in bicuspid aortic valve (BAV) progression is not fully understood. The aim of this study is to determine the significance of NFATC1 and NFATC4 gene expression for myocardial changes in children with BAV. Methods: In 47 children with BAV, the standard Doppler echocardiographic characteristics were detected, and the expression of the NFATC1 and NFATC4 genes was studied. Results: Posterior wall thickness in diastole (PWTd) and aortic valve peak pressure gradient (AoPPG) in BAV patients were significantly higher compared to healthy controls (PWTd median (min–max), 9 (7–10) mm vs. 7 (6–8) mm; and AoPPG median (min–max), 7.79 (2.98–15.09) mm Hg vs. 2.94 (2.42–3.72) mm Hg). The expression of the NFATC1 gene in BAV children was significantly higher compared to NFATC4 (NFATC1 median (min–max); 70.88 (8.79–106.51) e.u. vs. 7.72 (1.74–22.67) e.u., respectively p < 0.05). A significant correlation of NFATC1 expression with Ao found (R = +0.53, p < 0.05). In BAV patients with PWTd > 8 mm and Ao > 21 mm the NFATC1 expression was significantly higher compared to those with PWTd ≤ 8 mm and Ao ≤ 21 mm (NFATC1 median (min–max); 45.49 (5.01–101.52) e.u. vs. 15.53 (2.36–44.40) e.u., p < 0.05 and 81.11 (20.27–101.10) e.u. mm vs. 12.16 (2.40–45.49) e.u., p < 0.05, respectively). Conclusion: In children with BAV the high expression of the NFATC1 calcineurin signaling pathway gene is associated with elevated PWTd and Ao.Item Clinical variant of ossifying myositis in pediatric practice(2023-07) Сенаторова, Г.С.; Фролова, Т.В.; Сенаторова, А.В.; Кіхтенко, О.В.; Осман, Н.С.; Senatorova, H.; Frolova, T.; Senatorova, A.; Kikhtenko, E.; Osman, N.Introduction. Ossifying myositis is a pathological process in muscles characterized by the formation of ossification in soft tissues. At present, the etiological factors of the disease remain not fully elucidated. The triggering factors of the disease are considered to be traumatic injuries, invasive medical manipulations against the background of genetic predisposition. Aim. Invite attention of general practitioners and pediatricians to a rare disease, namely progressive ossifying fibrodysplasia in children and the peculiarities of its diagnosis. Results. The article presents a clinical case of progressive ossifying fibrodysplasia (Munchmeyer's disease) in a 4-year-old girl. At birth, the child was diagnosed with a foot deformity characteristic of this pathology (shortening of the first metatarsal finger, flexion-rotation contracture of both feet). The clinic of the disease manifested itself at the age of 3 years, when, after falling on the back, a dense formation was noticed in the area of the left shoulder blade. Half a year after the fall, swelling and pain appeared in the sacro-coccygeal region of the spine. The girl was consulted by an orthopedist, dermatologist, and oncologist. During the examination of the child, characteristic clinical features of progressive ossifying fibrodysplasia were revealed, namely, deformation and fixed position of the chest, tense neck muscles, sharp limitation of movements in all parts of the spine, limitation of bending in the left elbow joint, clinodactyly, valgus deformity of the big toes. During the ultrasound examination, the following changes were diagnosed: swelling of muscle tissue in the neck area, subscapular area on the left and sacrococcygeal joint; multiple hypoechoic formations of irregular shape, heterogeneous echo structure with hyperechoic inclusions with an acoustic shadow; a focal change in the muscle structure in the form of a loss of the characteristic pinnate structure of the perimysium. The diagnosis was confirmed histologically. No characteristic changes were found in clinical and biochemical studies. The girl is under supervision. No worsening of the child's condition has been recorded over the past four years.Item Problems of sleep disorders in children with paralytic syndromes(2023) Riga, Olena; Tkachenko, Olha; Tkachenko, YuliaItem Trajectories of serum 25(oh)d in children with paralytic syndromes when using vitamin d from different manufacturers and in different doses(2023-07) Ріга, Олена Олександрівна; Михайлова, О.В.; Michaylova, Olexandra; Riga, OlenaThe prevalence of vitamin D deficiency and insufficiency varies significantly in different subpopulations of children depending on age and diseases, and recommendations for their correction in children with paralytic syndromes are limited. Aim. Empirical determination of blood 25(OH)D trajectory in children with paralytic syndromes when using vitamin D from different manufacturers and in different doses. Material and methods. The 25(OH)D (ng/ml) of blood serum was analyzed by immune-enzymatic method "Labline-90" (Austria) with the test system "Monobind Inc." (ELISA, USA) in 77 children with paralytic syndromes aged 1-18 years, a repeat study after vitamin D3 supplementation was conducted in 36 children. The rate of increase in 25(OH)D concentration per month was calculated. Methods of descriptive statistics, non-parametric correlation analysis and Kaplan-Meier survival analysis were used with MedCalc Statistical Software (Belgium). This study was approved by the Ethics Committee (protocol No. 5, October 2021), which was conducted with the involvement of minor patients and did not contain measures that could harm their health. The research was carried out within the framework of the Department of Pediatrics of Kharkiv National Medical University "Medical and social aspects of adaptation of children with somatic pathology in modern conditions" (state registration number 0120U102471, 2020). Results. Vitamin D insufficiency was diagnosed in 17% of children with paralytic syndromes, and vitamin D deficiency in 73%, so daily doses of 2000-4000 IU of vitamin D3 from different manufacturers were recommended at the discretion of the parents for 6 months. In reality, children received doses from 500 to 5000 IU randomly, from 2 to 7 months. Doses were stratified as greater than 2000 IU and less than 2000 IU. If the child received a dose of 2000 IU or more, the rate of increase of 25(OH)D in the blood in children was 3.6 ng/ml per month, if the dose was less than 2000 IU - 1.6 ng/ml per month. Conclusions. Children with paralytic syndromes should be screened and monitored for serum 25(OH)D levels. With a serum 25(OH)D level of less than 20 ng/ml, daily administration of vitamin D3 in a dose of at least 2000 IU for at least 6 months allows reaching a 25(OH)D level of 30 ng/ml in most of them. Further large-scale studies are needed to supplement current recommendations for vitamin D3 supplementation in children with paralytic syndromes.Item Vitamin D status in children with paralitic syndroms(2023-09) Riga, Olena; Khaustov, Maxym; Mikhaylova, Aleksandra; Orlova, NataliaThe aim: Determination of serum 25(OH)D in the children with paralytic syndromes and its distribution depending on age, sex, taking anticonvulsant drugs, nutritional status for a period of one year (autumn-spring) of one center. Materials and methods: There were recruited of 77 children with paralytic syndromes and 73 health children for the same period aged from 1 till 18 years. The study included a scrutiny of medical history and analysis of medical documents, assessment of motor dysfunction by GMFCS, and nutritional status. Results: Among children with paralytic syndromes there were spastic tetraparesis 59.7%, malnutrition 92%, IV-V level of gross motor disfunction 80.5%, antiseizure medications 59.7% and cognitive impairment 77.9%. The variation of serum 25(OH)D is from 6.1 to 76.7 ng/mL with median 18.3 ng/mL in healthy children. The variation of serum 25(OH)D is from 2.2 to 83.0 ng/mL with median 14.8 ng/mL in children with paralytic syndromes (p=0.0103). Vitamin status among them is the following: insufficiency (21–29 ng/mL)–28.7% vs 16.8%; deficiency (<20 ng/mL)–56.1 vs 72.2% (p=0.0300). The 25.9% children with paralytic syndromes and those who have deficiency demonstrate severe deficiency (<10 ng/mL) compare 10.9% in healthy children (p=0.00189). There is a tendency to decrease of serum 25(OH)D in children with paralytic syndrome older 7 years. Conclusions: We failed to record a significant difference in the 25(ОН)D between males and females, between different level of GMFCS, and anticonvulsants using. Deficiency of vitamin D in 2.25 times higher in children with paralytic syndromes and severe malnutrition. Additional researches with specific items are need in perspective.