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不同类型心脏淀粉样变性的组织病理学和免疫组织化学特征。

Histopathological and Immunohistochemical Characteristics of Different Types of Cardiac Amyloidosis.

机构信息

Avtsyn Research Institute of Human Morphology, Petrovsky National Research Centre of Surgery, 117418 Moscow, Russia.

Institute of Medicine, Peoples' Friendship University of Russia named after Patrice Lumumba, 6 Miklukho-Maklaya St., 117198 Moscow, Russia.

出版信息

Int J Mol Sci. 2024 Oct 3;25(19):10667. doi: 10.3390/ijms251910667.

Abstract

Cardiac involvement is the most important factor determining prognosis in patients with systemic amyloidosis. This retrospective observational study of 98 patients with amyloidosis was undertaken to assess the amyloid types that are most likely to affect the heart, describe histopathological and clinical features of cardiac amyloidosis, and estimate the number of cases not diagnosed clinically prior to death. All cases were divided into two groups based on the method of examination. The first group included 46 patients with cardiac amyloidosis revealed via endomyocardial biopsies (EMBs), and the second group included 52 amyloidosis patients who did not undergo EMBs, in whom cardiac involvement was identified only at autopsy. The EMBs demonstrated that AL amyloidosis was detected in 21 (46%) specimens, ATTR amyloid in 24 cases (52%), and AA amyloid in 1 case (2%). The autopsy reports defined 15 (46%) cases of AL amyloidosis, 21 (40%) of ATTR and 16 (31%) of AA amyloidosis. It should be noted that a clinical diagnosis of ATTR amyloidosis was made only in 9.5% of patients from the autopsy group, suggesting that ATTR may be an underdiagnosed cause of heart failure in elderly patients. The most intense amyloid deposits were determined in biopsy and autopsy specimens of patients with AL kappa amyloidosis, underlying a poorer prognosis.

摘要

心脏受累是决定系统性淀粉样变性患者预后的最重要因素。本研究回顾性观察了 98 例淀粉样变性患者,旨在评估最有可能影响心脏的淀粉样变类型,描述心脏淀粉样变性的组织病理学和临床特征,并估计在死亡前临床未诊断的病例数。所有病例均根据检查方法分为两组。第一组包括 46 例经心内膜心肌活检(EMB)证实的心脏淀粉样变性患者,第二组包括 52 例未行 EMB 的淀粉样变性患者,仅在尸检中发现心脏受累。EMB 显示 21 例(46%)标本检测到 AL 淀粉样变性,24 例(52%)为 ATTR 淀粉样变性,1 例(2%)为 AA 淀粉样变性。尸检报告确定 15 例(46%)为 AL 淀粉样变性,21 例(40%)为 ATTR 淀粉样变性,16 例(31%)为 AA 淀粉样变性。值得注意的是,尸检组中仅 9.5%的患者被临床诊断为 ATTR 淀粉样变性,这表明 ATTR 可能是老年心力衰竭患者中诊断不足的原因。AL kappa 淀粉样变性患者的活检和尸检标本中淀粉样沉积物最丰富,预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e77/11476653/577d8213588a/ijms-25-10667-g001.jpg

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