CONGENITAL HEART DISEASES AND CONNECTIVE TISSUE DYSPLASIA CHILDREN OF SEMEY, THE DETERMINATION THE FREQUENCY
Introduction. The community of pathogenetic mechanisms of congenital heart diseases and connective tissue dysplasia connective tissue dysplasia defines increase in risk of their combinations. At the same time perhaps essential deterioration in the forecast of a current and conditions for correction of connective tissue dysplasia. So far poorly studied are an actual frequency and structure of combinations of these pathological states in population.
Research objective – to determine the frequency of signs connective tissue dysplasia in children with connective tissue dysplasia are living in Semey sity.
Materials and methods. the study was conducted on the basis of the medical center of the state medical University of Semey pediatric Department. The study was conducted in the period 2016-2018. This work is a retro-prospective, analytical experimental clinical and epidemiological study. 168 children aged 1 to 14 years were examined, including 84 children with connective tissue dysplasia and 84 healthy children of the control group. Definition of physical signs of connective tissue dysplasia at the first stage of diagnostics and assessment of visceral signs of systemic involvement at the second stage is carried out. The comparative analysis was carried out also taking into account connective tissue dysplasia option. For a statistical analysis of tables of an associativity used unilateral precise criterion of Fischer and criterion χ2.
Results of a research: Children with VPS and with connective tissue dysplasia had quite comparable frequency of phenotypical signs of the last. Significant excess of the frequenciest indexes over control group on the majority of signs was observed. Instrumental researches of heart showed very high frequency of signs of connective tissue dysplasia at children about connective tissue dysplasia. It is revealed that undifferentiated connective tissue dysplasia is almost inevitable accompanying syndrome at children with connective tissue dysplasia, being observed in 88,1% of cases.
The frequency of external and visceral signs of connective tissue dysplasia in children with congenital malformations was higher than in children with connective tissue dysplasia of 2-3 degrees. Severe dysplasia of connective tissue is characteristic of pulmonary artery stenosis, tetralogy of Fallot, transposition of CAPD, great vessels, coarctation of the aorta in the presence of bicuspidal aortic valve. Relative risk of birth in women with connective tissue dysplasia 2-3 degrees in children with congenital heart defects RR=12.5 ,2=98.23 (p<0.001)
Aizhan Z. Abylgazinova 1,
Aliya R. Alimbaeva 1,
Gulnara B. Tiorazova 1
1 Department of Pediatrics, Semey State Medical University.
Semey, the Republic of Kazakhstan
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18. Potter C.A., Armstrong-Wells J., Fullerton H.J. et al. Neonatal giant pial arteriovenous malformation: genesis or rapid enlargement in the third trimester. J Neurointerv Surg. 2009. Vol.1(2). P.151-153.
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21. Shivapour D.M., Erwin P., Kim E.Sh. Epidemiology of fibromuscular dysplasia: A review of the literature. Vasc Med. 2016. Vol.21(4). P.376-381.
22. Weiss K., Applegate C., Wang T., Batista D.A. Familial TAB2 microdeletion and congenital heart defects including unusual valve dysplasia and tetralogy of Fallot. Am J Med Genet A. 2015. Vol.167A(11). P.2702-2706.
23. Zhu X., Wang Y., Zhao G. et al. Phenotypic and genetic analysis of a child with blepharophimosis, ptosis, epicanthus inverses syndrome and tetralogy of Fallot. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2015. Vol.32(5). P.670-673.
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Абылгазинова А.Ж., Алимбаева А.Р., Тайоразова Г.Б. Частота признаков дисплазии соединительной ткани и врожденных пороков сердца у детей города Семей // Наука и Здравоохранение. 2018. 5 (Т.20). С. 145-152. Abylgazinova A.Z., Alimbaeva A.R., Tiorazova G.B. Congenital heart diseases and connective tissue dysplasia children of Semey, the determination the frequency. Nauka i Zdravookhranenie [Science & Healthcare]. 2018, (Vol.20) 5, pp. 145-152. Абылгазинова А.Ж., Алимбаева А.Р., Тайоразова Г.Б. Семей қаласының балаларындағы туа біткен жүрек ақауы мен дәнекер тін дисплазиясының жилігін анықтау // Ғылым және Денсаулық сақтау. 2018. 5 (Т.20). Б. 145-152.Related publications:
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