Kato Rie, Miyahara Hiroaki, Kawano Tatsuya, Matsuzuka Atsuko, Noda Kimiko, Izumi Tatsuro
Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan; Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan.
Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan.
Brain Dev. 2017 May;39(5):418-421. doi: 10.1016/j.braindev.2016.12.008. Epub 2017 Jan 12.
To elucidate the novel biological functions of heparan sulfate (HS) by clinic-pathologically studying a patient with paroxysmal atrioventricular (AV) block.
A long-surviving male patient with Sanfilippo syndrome type A presented with paroxysmal AV block at age 33years. He then survived another 2.5years after the onset of paroxysmal AV block and pacemaker implantation.
His cardiac histopathological examination at autopsy showed HS storage in the cardiac conduction system (CCS), especially in the atrioventricular node (AVN)-His bundle branches.
HS storage in the CCS might trigger AV block, arising from below the AVN-His bundle branches. This is the first description to indicate that HS might be an essential constituent of life-long CCS plasticity and that its storage in the CCS results in AV block.
通过对一名阵发性房室传导阻滞患者进行临床病理研究，阐明硫酸乙酰肝素（HS）的新生物学功能。
一名长期存活的A型Sanfilippo综合征男性患者，33岁时出现阵发性房室传导阻滞。在阵发性房室传导阻滞发作并植入起搏器后，他又存活了2.5年。
尸检时对其心脏进行组织病理学检查，结果显示心脏传导系统（CCS）中有HS蓄积，尤其是在房室结（AVN）-希氏束分支中。
CCS中HS蓄积可能引发房室传导阻滞，起源于AVN-希氏束分支以下。这是首次表明HS可能是CCS终身可塑性的重要组成部分，且其在CCS中的蓄积会导致房室传导阻滞。
