Senile systemic amyloidosis: are regional differences real or do they reflect different diagnostic suspicion and use of techniques?

Senile systemic amyloidosis: are regional differences real or do they reflect different diagnostic suspicion and use of techniques?
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DOI:
10.3109/13506129.2012.674074
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发表时间:
2012-06-01
影响因子:
5.5
通讯作者:
Falk, Rodney H.
Falk, Rodney H.
中科院分区:
医学2区
文献类型:
--
作者:
Falk, Rodney H.

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在老年患者尸检时,心脏中淀粉样沉积物来源于甲状腺素运载蛋白。大量沉积,引起老年性系统性淀粉样变性(SSA),被认为是相对罕见的,报告的系列表明,SSA是相当不常见的淀粉样轻链(AL)淀粉样变性。本文回顾了甲状腺素运载蛋白衍生的心脏淀粉样蛋白的尸检患病率,讨论了是否存在地区或种族差异的明确证据,并认为SSA的报告患病率明显低于该疾病的真实患病率,该疾病可能与AL淀粉样变性一样常见或更常见。
Amyloid deposits in the heart derived from transthyretin are commonly seen at autopsy in elderly patients. Massive deposition, causing senile systemic amyloidosis (SSA), is considered to be relatively uncommon and reported series suggest that SSA is considerably less common than amyloid light-chain (AL) amyloidosis. This article reviews the autopsy prevalence of transthyretin-derived cardiac amyloid, addresses whether there is definitive evidence for regional or ethnic differences and argues that the reported prevalence of SSA significantly underrepresents the true prevalence of the disease which may be as common, or commoner, than AL amyloidosis.