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KMID : 0357820090330010019
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Korean Journal of Legal Medicine
2009 Volume.33 No. 1 p.19 ~ p.26
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Pathogenesis of Sudden Manhood Death Syndrome
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Huh Gi-Yeong
Kim Ye-Won
Kim Kwang-Hoon
Ahn Yong-Woo
Jo Gam-Rae
Jeong Seong-Muk
Lee Sang-Yong
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Abstract
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Sudden manhood death syndrome (SMDS) is a disorder found in southeast Asia, particularly Thailand, Philippines and Japan, which causes sudden cardiac death during sleep. In Korea, SMDS cases have been occasionally encountered in forensic autopsy practice. However, the incidence and pathogenesis has been rarely studied. This study to review chronologically the proposed pathogenesis of SMDS; pathology of cardiac conduction system, sleeping and breathing disorder, K+ and thiamine deficiency, mental stress, testosterone, hyperlipidemia, and narrow circumference of coronary artery. Brugada syndrome and SMDS are phenotypically, genetically and functionally the same disorder and Brugada syndrome has been a subject of intensive study since its early description in 1992. While Brugada syndrome is an inherited cardiac disorder caused by mutations in the SCN5A gene encoding the cardiac sodium channel alpha-subunit. less than 20% of its patients are known to be associated with SCN5A mutations. Moreover, the postmortem molecular screening of SCN5A mutations on formalin fixed paraffin embbeded cardiac tissues from SMDS cases has not been rewarding due to technical problems. The role of structural heart disease and sodium channel dysfunction in the induction of electrical instability in SMDS and Brugada syndrome is still debatable.
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KEYWORD
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sudden manhood death syndrome, brugada syndrome, pathogenesis, SCN5A
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