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

Takeichi和Fujita（2006）提出了心源性猝死的新概念Pokkuri Death Syndrome（PDS），其可能是由于早期2/3的PDS病例中观察到的与残余高脂蛋白血症相关的强烈冠状动脉痉挛引起的，与美国两家研究机构进行了心源性猝死与残余脂蛋白（RLP）关系的国际合作研究。其中一个是与CVPath（国际病理学注册）的Virmani博士，法医学研究人员，对76例严重冠状动脉粥样硬化的心脏性猝死病例进行血浆分析。但是，在美国，由于心脏性猝死病例尸检时的死亡时间较长，其死后血浆变化太严重，无法分析脂质和脂蛋白。另一个是VA-HDL干预试验（VA-HIT）中的血脂和脂蛋白分析（2531例使用和不使用吉非罗齐的男性患者）与Tufts Univ.的Schaefer博士关于与心血管（CV）终点发生的关系，如致死性或非致死性MI、卒中等。通过比较日本和美国病例，VA-Hit研究表明，在日本，RLP-TG是与PDS一样的CV终点的强预测因子，但apoB和LDL-C并非如此。由于1/3的PDS病例与高脂血症无关，因此作为与蒙特利尔大学Brugada博士的第三次国际合作，对PDS病例进行了与Brugada综合征相关的SCN 5A的遗传分析，加拿大PDS患者中有一例存在类似Brugada综合征的SCN 5A基因突变。目前正在对这些病例进行进一步的基因分析。

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.