2011-08-012024-05-13https://scholars.lib.ntu.edu.tw/handle/123456789/648344摘要：兒童猝死遠比成人期少見。文獻報告提出之年發生率大約為1.3-4.3/10萬兒童人口。相較於成人的發生率可高達每千人即有1人，兒童猝死的發生率顯然較低。在最近的一篇來自美國Oregon的統計報告，總人口群猝死率為60人/10萬人口，而兒童猝死有7.5/10萬人。此外，兒童猝死的預後亦較差，8％的成人猝死能被救活，而兒童猝死被救活的概率卻趨近於零。此外，因在嬰兒期（0-1歲）其猝死之病因與較大兒童並不同，可因此在醫學上另分類為「嬰兒猝死症」。嬰兒猝死症年發生率在西方國家普遍比東方國家高，即使在西方國家推廣「Back to Sleep」等運動後，其發生率仍大致高於東北亞國家。相對於嬰兒期猝死的觀察，兒童猝死之流行病學資料相當有限，亦無亞洲國家之相關報告。台灣的總人口為2千3百萬，其中560萬為20歲內之兒童。1995年台灣之全國健康保險制度成立，提供全民健康醫療服務。而其診斷與相關資料亦經收錄成為全國國民健康資料庫。我們由此資料庫之資料作初步的分析，我們發現兒童（1-18歲）年猝死率為2.7/10萬人口，而嬰兒猝死率為0.36/1000活產兒。為了解這些數字是否隱含我國健康政策之完整性健全與否。我們亦由內政部之相關資料庫及美國之相關資料出版物導出各項兒童健康指標。我們的分析發現，我國兒童健康指標與美國是大致相當的。這些數字顯示我們的嬰兒猝死率與兒童猝死之概率的確可能低於西方國家。其原因有待釐清。猝死的原因在成人與兒童是大不相同的。約半數之兒童期猝死可能為心因性猝死。排除結構異常之心臟疾病，最主要的猝死病因應為因離子通道異常而造成的primary cardiac arrhythmia。因此我們的研究假設為(1)病人若有心臟離子通道異常有極高的概率在兒童期或50歲內之成人期發生猝死（可能死亡或經急救救回，aborted sudden death）(2)全國健保資料庫可提供粗估之我國50歲內總人口及各年齡層人口之猝死率；及(3)此外，另外若能由醫院之資料庫進一步分析可再提供50歲內總人口及各年齡層之猝死病因，臨床表現及預後之資料。且由確立為心因性（非缺氧性心臟病）之活存者或即其家屬亦可再進一步檢測其基因學變化，以得知其序列基因突變及離子通道病變之genotype-phenotype相關。如此之研究設計，我們可得到(1)許多重要的猝死流行病學資料以提供國家衛生政策建立之重要的指標與政策制立之根據；(2)亦可知道我國常見之猝死相關基因突變；及(3)猝死之預後及其臨床表現亦可提供設立體內去顫器植入指引之重要資料。<br> Abstract: In pediatric population, Sudden death (SD) is relatively uncommon. The incidence rate has been estimated to be 1.3-4.3 per 100,000 person-years in the pediatric population beyond infancy. The annual incidence of SD in the general population is cited as 1 in a 1,000. In a recent report from the Oregon, the pediatric SD constituted only 2.8% of all SD of the population and the annual incidence rate per 100,000 children was 7.5 (95% CI 5.1–10.5), compared with 60/100,000 for all ages. In contrast to an adult survival rate of 8%, none of the children survived to be discharged from the hospital. In infants, the incidence of SD is generally considered to be higher than that in other pediatric populations. With distinct underlying mechanisms, sudden infant death syndrome (SIDS) has been recognized as a distinct disease entity. The incidence had been relatively high, but now, after the launching of educational campaigns such as the “back to sleep” act, it has decreased from 2.69 to around 0.5 per 1,000 live births. In Asian populations, the incidence of SD in infants is in general lower than that in Western populations. However, such epidemiological data for SD in pediatric populations beyond infancy are still very limited. The proportion of SD accounted for the SD in the general population in Asia remains unknown.In Taiwan, the general population and the pediatric population (0-20 years) is around 23 and 5.6 millions, respectively. A nationwide National Health Insurance (NHI) program was implemented in Taiwan in 1995 and it covers the health care of >98% of the population. The medical information for each patient has been collected and constitutes the NHI database. From this database, we have conducted some preliminary studies. We found that the annual incidence rate was 2.7 per 100,000 children in the pediatric population aged from 1 to 18 years of age and an incidence of 0.36/1,000 live births in infants . The preliminary scan also revealed that the child health indexes of Taiwan are quite comparable to those in US.The cardiac causes of SD in children without structural heart diseases include mainly primary cardiac arrhythmias due to channelopathy (long QT syndrome, catecholinergic polymorphic ventricular tachycardia, Brugada syndrome, short QT syndrome, and arrhythmogenic right ventricular dysplasia).We therefore hypothesize that 1) patients with underlying cardiac channelopathy may encounter SD or SD successfully resuscitated (aborted SD) during the childhood or young adulthood before the age of 50 years 2) National database after data validation may provide the most approximate estimate for the SD, 3) An institutional database may provide the spectrum of underlying diseases for SD, from the aborted SD and the family members of the victim of SD, genetic studies for the underlying channelopathy can be performed.Aims: The specific aims of this study are 1) by using the NHI national data base, to have an epidemiological scan for the SD in Taiwan country, an Asian population cohort with well established health care policy: we will have the incidence of SD in each age-population, i.e., infants, pediatric population 1-20 years, young adults 20- 30 years and middle age adults 30-50 years of age, and 2) by using additional institutional approach, to search for the clinical manifestations, underlying diseases, and to define the associated channelopathy in those with confirmed unexplained SD or arrhythmic SD.Study design:Part I. Incidence and international comparison: this part will be conducted on the NHI database 2000-6Part II. Spectrum of underlying diseases and outcomes: this part will be conducted on Institutional database 1990-2009.Part III. Genomic studies of those with unexplained SD: this part will be conducted on the study cohort identified from part II study, the aborted SD and the family members of aborted SD and SD victims.Predictive outcome: After the completion of the study, the results will provide us the information regarding 1) the incidence of SD in general population and subgroup in age, which will be very important data for the National Health Policy making or even the policy making regarding the intensity of AED or other resuscitation team build-up; 2) genetic mutations for cardiac channelopathy which is often associated with racial-related differences; and 3) outcomes of the SD which will be important for setting up the guidelines for those non-ischemic SD regarding the indication of intracardiac cardiac defibrillator as primary prevention.Potential difficulties and Solutions1.For the NHI database study, the most difficult issues will be the lack of link to the individual patient data. This will bring up the uncertainty of diagnosis. But, this will be at least in part solved by appropriate data validation.2.For the Institutional-based study, the potential difficulty will be the genomic study. SD is a very painful experience to the family members. Therefore, the contact for the family members of SD victims will be challenging. This problem may be solved by some supporting groups, such as Taiwan Cardiac Children Foundation. These organizations may play a more neutral role in helping the in-shock family members to face the SD issue again.猝死兒童