US-Japan comparative study of the sudden cardiac death of young males-pokkuri death syndrome (PDS).

Pokkuri死亡综合征(PDS)是由Takeichi和Fujita(2006)提出的心脏性猝死的新概念，其原因可能是2/3的PDS患者普遍存在与残余高脂蛋白血症相关的强烈冠脉痉挛。其中一项是与法医研究员、CVPath(国际病理学注册中心)的Virmani博士一起对76例患有严重冠状动脉粥样硬化的心脏性猝死病例进行血浆分析。但由于美国心脏性猝死病例尸检时间隔时间较长，故对此类研究而言，其血浆的死后变化过于严重，难以进行脂蛋白分析。另一项是与Tuft大学的Schaefer博士一起进行的VA-高密度脂蛋白干预试验(VA-HIT)(2531名服用和不服用吉非罗齐的男性患者)的血脂和脂蛋白分析。通过比较日本和美国的病例，VA-HIT研究表明，在日本，RLP-TG与PDS一样是心血管终点的有力预测因子，但不能同时预测apoB和LDL-C。由于1/3的PDS病例与高脂血症无关，与Brugada综合征相关的SCN5A基因分析是第三次与加拿大蒙特利尔大学的Brugada博士合作进行的。PDS患者存在类似Brugada综合征的SCN5A基因突变。在这些病例中，进一步的基因分析正在进行中。

The new concept of Pokkuri Death Syndrome (PDS) for sudden cardiac death was proposed by Takeichi and Fujita (2006), which may be caused by the strong coronary spasm associated with remnant hyperlipoproteinemia observed prevalently in 2/3 of PDS cases.The international collaboration research on the relationship between sudden cardiac death and remnant lipoproteins (RLP) were performed with the two US research institutions. One was with Dr. Virmani of CVPath (International Registry of Pathology), a forensic medicine researcher, on plasma analysis of 76 sudden cardiac death cases with severe coronary atherosclerosis. But the postmortem alteration of plasma in sudden cardiac death cases in USA was too severe to analyze lipid and lipoproteins because of the long postmortem interval at the time of autopsy in these cases for this kind of study. The other was the lipid and lipoprotein analysis in VA-HDL Intervention Trial (VA-HIT) (2531 male patients with and without gemfibrozil) with Dr. Schaefer of Tufts Univ. on the relationship to the occurrence of cardiovascular (CV) endpoints such as fatal or non-fatal MI, stroke etc. By comparing the Japanese and US casese, VA-Hit study showed that RLP-TG was a strong predictor of CV endpoints as PDS in Japan, but not apoB and LDL-C as well. As 1/3 of PDS cases were not associated with hyperlipidemia, the genetic analysis in PDS cases on SCN5A related with Brugada Syndrome was performed as the third international collaboration with Dr. Brugada at Montreal Univ., Canada. The cases with SCN5A gene mutations similar to Brugada Syndrome were found in PDS cases. Further genetic analysis is on going in these cases.