[Morphology of the myocardium of the interventricular septum in children with hypertrophic cardiomyopathy].

Q4 Medicine
T V Sukhacheva, R A Serov, D A Malenkov, M I Berseneva, L A Bokeria
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引用次数: 0

Abstract

Objective: To carry out a comparative analysis of the morphology of the interventricular septum (IVS) myocardium in children with hypertrophic cardiomyopathy (HCM) and without cardiovascular pathology.

Material and methods: A study of myocardial biopsies of the IVS in children with HCM (n=18, 1.2-17 years) and children without cardiovascular pathology (n=11, 1-16 years) was carried out. The volume of interstitial tissue in the IVS myocardium was determined, a morphometric study of the size of cardiomyocytes (CMCs), the myofibrillogenesis level and the ploidy of CMCs was carried out, the ultrastructure of the CMCs was studied, and the localization of the gap junction protein, connexin43 (Cx43), was revealed by immunohistochemistry.

Results: The proportion of interstitial tissue in the myocardium of children with HCM was 9-10% and did not differ from its proportion in the myocardium of children in the control group. The diameter of the CMCs of the IVS in children with HCM reached the limit of ontogenetic growth and exceeded the parameters of the control group (average 18.9±5.7 µm vs 9.3±4.4 µm). CMCs ploidy in children with HCM was 2 times higher than CMCs ploidy in control patients (5.3c vs 2.7c). In the myocardium of children with HCM, the assembly of myofibrils most actively occurred in small CMCs. At the ultrastructural level, signs of immaturity of the contractile apparatus and intercalated discs of the CMC in HCM were demonstrated. In the myocardium of children with HCM, Cx43-containing gap junctions were more often located on the lateral surfaces of the CMC than in the myocardium of the control group.

Conclusion: In children with HCM, a morphological picture of an increase in the size of the CMCs and their ploidy during accelerated ontogenetic development was demonstrated in combination with ultrastructural signs of immaturity of the contractile apparatus and intercalated discs and the lack of growth of interstitial tissue of the IVS myocardium compared with patients in the control group.

[肥厚型心肌病患儿室间隔心肌形态学研究]。
目的:对肥厚性心肌病(HCM)患儿与无心血管病变患儿室间隔(IVS)心肌形态进行比较分析。材料与方法:对HCM患儿(n=18, 1.2 ~ 17岁)和无心血管病理患儿(n=11, 1 ~ 16岁)的IVS心肌活检进行研究。测定IVS心肌间质组织体积,形态学研究心肌细胞(CMCs)大小、肌原纤维形成水平和CMCs倍性,研究CMCs超微结构,免疫组化检测间隙连接蛋白connexin43 (Cx43)的定位。结果:HCM患儿心肌间质组织比例为9 ~ 10%,与对照组患儿心肌间质组织比例无显著差异。HCM患儿IVS的cmc直径达到了个体生长的极限,超过了对照组的参数(平均18.9±5.7µm vs 9.3±4.4µm)。HCM患儿的cmc倍性是对照组的2倍(5.3c vs 2.7c)。在HCM患儿心肌中,肌原纤维的组装最活跃地发生在小的cmc中。在超微结构水平上,HCM显示了CMC的收缩器和嵌盘不成熟的迹象。在HCM患儿心肌中,与对照组相比,含cx43的间隙连接更多地位于CMC的外侧表面。结论:在HCM患儿中,与对照组患者相比,在个体发育加速的过程中,CMCs的大小和倍性增加,并伴有收缩器和间插盘不成熟的超微结构征象,以及IVS心肌间质组织生长缺乏。
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来源期刊
Arkhiv patologii
Arkhiv patologii Medicine-Pathology and Forensic Medicine
CiteScore
0.90
自引率
0.00%
发文量
55
期刊介绍: The journal deals with original investigations on pressing problems of general pathology and pathologic anatomy, newest research methods, major issues of the theory and practice as well as problems of experimental, comparative and geographic pathology. To inform readers latest achievements of Russian and foreign medicine the journal regularly publishes editorial and survey articles, reviews of the most interesting Russian and foreign books on pathologic anatomy, new data on modern methods of investigation (histochemistry, electron microscopy, autoradiography, etc.), about problems of teaching, articles on the history of pathological anatomy development both in Russia and abroad.
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