Cardiac Amyloidosis in Aging African Americans

描述(来自应用程序的逐字)：如果与访问相关的因素 如果不考虑护理，#年心血管疾病死亡率的增加 非裔美国人相对于其他群体来说是由于 一些疾病，一种对疾病性质不同的心脏反应 影响所有民族，对治疗反应相对较差 充血性心力衰竭一种未确诊的，共存的，相对流行的， 难治性心肌病可能是部分原因。晚些 起病的淀粉样心肌病在非裔美国人中的发病率是美国人的四倍 而不是白种人。然而，它会导致充血性心力衰竭和心律失常。 这些特征是相对非特异性的，临床诊断也不是 总是显而易见的。地高辛和钙通道阻滞剂对慢性阻塞性肺疾病患者有毒性作用 因此，淀粉样蛋白治疗其他原因的伴发性心脏病可能 此外，淀粉样心脏病的误诊可能会导致 可能是有害的治疗。未被识别的淀粉样变性，在60岁以上的人中， 可能导致在充血性心力衰竭的研究中看到的一些顽固性 心力衰竭和较高的心血管发病率和死亡率 非裔美国人。 我们的方案研究了一种由基因决定的迟发性淀粉样变性。 由于血清蛋白中第122位的Ile取代Val 转甲状腺素(TtR)。大约4%的非裔美国人是杂合子 老年后有绝对风险发生解剖性淀粉样沉积的等位基因 60导致154,000名非洲裔美国人患有不同程度的心脏病 淀粉样变性。在与两项心血管风险研究的合作中 在社区(ARIC和CFIS)，有一群非洲裔美国人 5200，我们将检验导致淀粉样变的杂合性假说 等位基因与心脏淀粉样变性的临床证据和 死亡率的相关增加。我们还将评估等位基因在 通过确定心脏病在一群人中的患病率来确定临床心脏病 60岁以上的非裔美国退伍军人，他们被认为患有心脏病， 尽管他们的提供者没有考虑将淀粉样变性作为一种特殊的 诊断。我们将描述迟发性心脏病的自然病史。 非裔美国人的淀粉样变性在心血管疾病发病中的作用 和死亡率，并确定支持的指导方针 目前的治疗方法和可用的具体治疗方法。

DESCRIPTION (Verbatim from the application): If factors related to access to care are not considered, the increased mortality from cardiovascular disease in African-Americans relative to other groups is due to the increased frequency of some diseases, a qualitatively different cardiac response to disorders affecting all ethnic groups and a relatively poor response to treatment of congestive heart failure. An undiagnosed, coexistent, relatively prevalent, treatment-resistant cardiomyopathy is a possible partial explanation. Late onset amyloidotic cardiomyopathy is fourfold more common in African-Americans than Caucasians. It causes congestive heart failure and arrhythmias, however these features are relatively non-specific and the clinical diagnosis is not always obvious. Digoxin and calcium channel blockers are toxic in patients with amyloid, thus, treatment of concomitant heart disease of other etiologies may be compromised; moreover misdiagnosis of amyloid heart disease may result in possibly harmful therapy. Unrecognized amyloidosis, in individuals over age 60, could contribute to some of the refractoriness seen in studies of congestive heart failure and to the higher cardiovascular morbidity and mortality in African-Americans. Our proposal examines a genetically determined form of late-onset amyloidosis due to a substitution of ILE for VAL at position 122 in the serum protein transthyretin (TTR). Approximately 4% of African-Americans are heterozygous for the allele that has an absolute risk for anatomic amyloid deposition after age 60 resulting in 154,000 African-Americans with some degree of cardiac amyloidosis. In a collaborative effort with two studies of cardiovascular risk in the community (ARIC and CFIS), with a combined African-American cohort of 5200, we will test the hypothesis that heterozygosity for the amyloidogenic allele is associated with clinical evidence of cardiac amyloidosis and a related increase in mortality. We will also assess the role of the allele in clinical heart disease by determining its prevalence in a cohort of African-American veterans, over 60, who are recognized as having heart disease, although their providers have not considered amyloidosis as a specific diagnosis. We will characterize the natural history of late onset cardiac amyloidosis in African-Americans, define its role in cardiovascular morbidity and mortality in this ethnic group and define guidelines for supportive treatment at present and specific therapy when available.