Сardiopylmonary transthyretin amyloidosis

Z. Gioeva, A. Ephiev
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Abstract

Wild type transthyretin amyloidosis (ATTRwt) affects a number of target organs, most commonly the heart. Over the last years, autopsy findings revealed that the lungs are involved in the pathological process more frequently than diagnosed in premortem cases. Due to the nonspecific nature of ATTRwt clinical manifestations and the presence of multiple concomitant conditions in elderly people, the majority of patients are diagnosed at a late stage or the disease is overlooked. As a result, ATTRwt has emerged as a previously underestimated cause of morbidity and mortality in the older age group. This article presents a clinical case report describing an 88-year-old female patient with generalized transthyretin amyloidosis characterized by predominant cardiac and pulmonary involvement. The paper elucidates the difficulties associated with premortem diagnosis of this type of amyloidosis which include the absence of amyloid deposits in biopsy specimens from the rectum and subcutaneous fat. The specific information in this case report is focused on the discrepancy between clinical and autopsy diagnosis. Keywords: wild type transthyretin amyloidosis, cardiac amyloidosis, pulmonary amyloidosis, senile amy-loidosis, chronic heart failure
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С心外膜转甲状腺素淀粉样变性
野生型甲状腺转蛋白淀粉样变(ATTRwt)影响许多靶器官,最常见的是心脏。在过去的几年里,尸检结果显示,肺部参与病理过程比死前诊断的病例更频繁。由于ATTRwt临床表现的非特异性,且在老年人中存在多种合并症,多数患者诊断较晚或忽视该病。因此,attrt已成为以前被低估的老年人群发病和死亡的原因。本文提出一个临床病例报告,描述了一个88岁的女性患者广泛性甲状腺素转淀粉样变,主要表现为心脏和肺部受累。本文阐明了与这种淀粉样变的死前诊断相关的困难,其中包括直肠和皮下脂肪活检标本中缺乏淀粉样沉积。本病例报告的具体信息集中在临床和尸检诊断之间的差异。关键词:野生型转甲状腺蛋白淀粉样变,心脏淀粉样变,肺淀粉样变,老年性淀粉样变,慢性心力衰竭
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来源期刊
Clinical and Experimental Morphology
Clinical and Experimental Morphology Biochemistry, Genetics and Molecular Biology-Cancer Research
CiteScore
0.60
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0.00%
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18
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