van Ham Willem B, Meijboom Esmeralda E M, Ligtermoet Merel L, Nikkels Peter G J, van Veen Toon A B
Department of Medical Physiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands.
Department of Pathology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands.
J Cardiovasc Dev Dis. 2023 Aug 19;10(8):354. doi: 10.3390/jcdd10080354.
The development of the normal human heart, ranging from gestational age to the mature adult heart, relies on a very delicate and timely orchestrated order of processes. One of the most striking alterations in time is the gradual extinction of the ability for cardiomyocytes to proliferate. Once passing this event, cardiomyocytes grow and increase in contractile strength by means of physiological hypertrophy. This process, importantly, seems to depend on an adequate development of electromechanical coupling that is achieved by the appropriate formation of the intercellular junction named the intercalated disc (ICD). In this report, we describe two sudden death cases of young and apparently healthy-born individuals without external abnormalities compared to an age-matched control. Histological examination, including the comparison with the age-matched and histology-matched controls, showed a disturbed formation of the protein machinery composing the electromechanical junctions at the ICD and an increased nuclei count for both patients. As a cause or consequence, cardiomyocytes in both sudden death cases showed signs of a delayed developmental stage, presumably resulting in an exaggerated degree of hyperplasia.
从胎龄到成熟的成人心脏，正常人类心脏的发育依赖于一系列非常精细且适时协调的过程。时间上最显著的变化之一是心肌细胞增殖能力的逐渐丧失。一旦经历这一过程，心肌细胞通过生理性肥大生长并增强收缩力。重要的是，这一过程似乎依赖于通过名为闰盘（ICD）的细胞间连接的适当形成而实现的机电耦合的充分发育。在本报告中，与年龄匹配的对照组相比，我们描述了两例年轻且看似健康出生、无外部异常的猝死病例。组织学检查，包括与年龄匹配和组织学匹配的对照组进行比较，显示构成ICD处机电连接的蛋白质机制形成紊乱，且两名患者的细胞核计数增加。作为原因或结果，两例猝死病例中的心肌细胞均显示出发育阶段延迟的迹象，推测这导致了过度增生。
