日本经甲状腺素酶阳性心脏活检病例数量急剧增加:淀粉样变分型诊断的全国性病理会诊获得的证据。

IF 5.2 2区 医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY Amyloid-Journal of Protein Folding Disorders Pub Date : 2023-09-01 DOI:10.1080/13506129.2023.2180334
Hironobu Naiki, Aina Yamaguchi, Yoshiki Sekijima, Mitsuharu Ueda, Kenichi Ohashi, Kinta Hatakeyama, Yoshihiko Ikeda, Yoshinobu Hoshii, Yukako Shintani-Domoto, Aya Miyagawa-Hayashino, Hanako Tsujikawa, Jin Endo, Tomio Arai, Yukio Ando
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引用次数: 2

摘要

背景:在2019年、2020年和2022年,日本政府批准了他法底斯和两种技术- scintix用于甲状腺素淀粉样蛋白转移(ATTR)型心肌病,并公布了他法底斯治疗的患者标准。2018年,我们在全国范围内开展了淀粉样变病理会诊。目的:探讨批准他非他司及超声显像对ATTR型心肌病诊断的影响。方法:10家机构参与淀粉样变性病理会诊,共享兔抗κ116-133、抗λ118-134、抗转甲状腺视黄酮115-124抗体。当无法通过免疫组织化学进行分型诊断时,进行蛋白质组学分析。结果:在2018年4月至2022年7月收到的5400例咨询病例中,4420例刚果红阳性病例中有4119例通过免疫组织化学确定了淀粉样变的类型。AA、ALκ、ALλ、ATTR、Aβ2M和其他基因的发生率分别为3.2、11.3、28.3、54.9、0.6和1.8%。2208例心脏活检病例中,1503例ATTR阳性。过去12个月的总病例数和atr阳性病例数分别是前12个月的4.0倍和4.9倍。结论:他法非底斯和技术显像的批准提高了人们对ATTR心肌病的认识,导致ATTR阳性心脏活检病例激增。
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Steep increase in the number of transthyretin-positive cardiac biopsy cases in Japan: evidence obtained by the nation-wide pathology consultation for the typing diagnosis of amyloidosis.

Background: In 2019, 2020 and 2022, the Japanese Government approved the use of tafamidis and two technetium-scintigraphies for transthyretin amyloid (ATTR) cardiomyopathy, and announced the patient criteria for tafamidis therapy. In 2018, we had started a nation-wide pathology consultation of amyloidosis.

Objective: To reveal the impact of approval of tafamidis and technetium-scintigraphy on the diagnosis of ATTR cardiomyopathy.

Methods: Ten institutes participated in this study on the pathology consultation of amyloidosis and shared rabbit polyclonal anti-κ116-133, anti-λ118-134, and anti-transthyretin115-124 antibodies. Proteomic analysis was performed when the typing diagnosis by immunohistochemistry was unavailable.

Results: Out of 5400 consultation cases received from April 2018 to July 2022, the type of amyloidosis by immunohistochemistry was determined in 4119 of the 4420 Congo-red positive cases. The incidences of AA, ALκ, ALλ, ATTR, Aβ2M and others were 3.2, 11.3, 28.3, 54.9, 0.6 and 1.8%, respectively. Out of 2208 cardiac biopsy cases received, 1503 cases were ATTR positive. There were 4.0 and 4.9 times more total cases and ATTR-positive cases, respectively, in the last 12 months as compared to the first 12 months.

Conclusions: The approval of tafamidis and technetium-scintigraphy raised the awareness of ATTR cardiomyopathy, leading to an upsurge in ATTR-positive cardiac biopsy cases.

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来源期刊
Amyloid-Journal of Protein Folding Disorders
Amyloid-Journal of Protein Folding Disorders 生物-生化与分子生物学
CiteScore
10.60
自引率
10.90%
发文量
48
审稿时长
6-12 weeks
期刊介绍: Amyloid: the Journal of Protein Folding Disorders is dedicated to the study of all aspects of the protein groups and associated disorders that are classified as the amyloidoses as well as other disorders associated with abnormal protein folding. The journals major focus points are: etiology, pathogenesis, histopathology, chemical structure, nature of fibrillogenesis; whilst also publishing papers on the basic and chemical genetic aspects of many of these disorders. Amyloid is recognised as one of the leading publications on amyloid protein classifications and the associated disorders, as well as clinical studies on all aspects of amyloid related neurodegenerative diseases and major clinical studies on inherited amyloidosis, especially those related to transthyretin. The Journal also publishes book reviews, meeting reports, editorials, thesis abstracts, review articles and symposia in the various areas listed above.
期刊最新文献
International prevalence of transthyretin amyloid cardiomyopathy in high-risk patients with heart failure and preserved or mildly reduced ejection fraction. No body fits in the test tube - the case of transthyretin. T2-relaxometry in a large cohort of hereditary transthyretin amyloidosis with polyneuropathy. Possible transmission of leukocyte chemotactic factor 2 amyloidosis after interpopulational liver transplantation. Double pathogenic variant in an ATTRv patient with mixed phenotype.
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