俄罗斯左心室肥大老年患者中 ATTR 淀粉样变性患病率的原始数据。

IF 0.5 4区医学 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

Kardiologiya Pub Date : 2024-04-30 DOI:10.18087/cardio.2024.4.n2611

T V Nikiforova, K V Charaya, D Yu Shchekochikhin, Z M Magomedova, M S Enokyan, A N Volovchenko, A H Khamzatkhanova, T A Starovoytova, A A Bogdanova, A V Karalkin, S P Pasha, E S Pershina, A E Grachev, I V Zhirov, D A Andreev

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引用次数: 0

摘要

目的：估算年龄为65岁、室间隔（IVS）肥厚≥14 mm的患者中由转甲状腺素淀粉样变性（ATTR）和免疫球蛋白轻链（AL）淀粉样变性引起的淀粉样心肌病（CM）的患病率：自2023年1月至8月，共纳入60例患者（平均年龄为7.2±7.3岁，男性34例（56.67%））。符合纳入标准的患者均接受了超声心动图检查，包括心肌纵向应变测定、99m锝-吡咯伏特心肌闪烁扫描、心肌单光子发射计算机断层扫描、脑钠肽 N 端片段和肌钙蛋白 I 的测定，以及血清和尿液蛋白的免疫化学检查和游离轻链的测定。如果闪烁扫描出现 2 级和 3 级放射性药物摄取，则进行分子遗传学研究，以鉴别诊断野生型转甲状腺素淀粉样变性（wtATTR）和遗传性/变异型（hATTR）ATTR-CM：根据99m锝-pyrfotech心肌闪烁扫描数据，5例（8.3%）患者在无单克隆分泌的情况下出现3级摄取，6例（10%）患者在无单克隆分泌的情况下出现2级放射性示踪剂摄取。有5例（8.3%）患者的骨髓瘤并发AL淀粉样变性和原发性AL淀粉样变性：结论：在年龄≥65岁、IVS肥大≥14毫米的患者中，20%的病例（12例）检测到淀粉样CM，包括5例（8.3%）AL淀粉样变性和7例（11.7%）ATTR淀粉样变性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Primary Data on ATTR-Amyloidosis Prevalence Among Elderly Patients With Left Ventricular Hypertrophy in Russia.

Aim: To estimate the prevalence of amyloid cardiomyopathy (CM) caused by transthyretin amyloidosis (ATTR) and immunoglobulin light chain (AL) amyloidosis among patients aged >65 years with interventricular septal (IVS) hypertrophy of ≥14 mm.

Material and methods: From January through August 2023, 60 patients (mean age 7.2±7.3 years, 34 (56.67%) men) were enrolled. Patients meeting the inclusion criteria underwent an echocardiographic study with determining the myocardial longitudinal strain, myocardial scintigraphy with 99mTc-pyrfotech, myocardial single-photon emission computed tomography, measurement of N-terminal fragment of brain natriuretic peptide and troponin I, and the immunochemical study of serum and urine proteins with measurement of free light chains. In the presence of grades 2 and 3 radiopharmaceutical uptake according to scintigraphy, a molecular genetic study was performed for differential diagnosis of wild-type transthyretin amyloidosis (wtATTR) and hereditary/variant (hATTR) ATTR-CM.

Results: According to data of myocardial scintigraphy with 99mTc-pyrfotech, grade 3 uptake in the absence of monoclonal secretion was detected in 5 (8.3%) cases and grade 2 radiotracer uptake in the absence of monoclonal secretion was detected in 6 (10%) patients. Myeloma complicated by AL amyloidosis and primary AL amyloidosis were found in 5 (8.3%) patients.

Conclusion: Among patients aged ≥65 years with IVS hypertrophy ≥14 mm, amyloid CM was detected in 20% of cases (12 patients), including 5 cases (8.3%) of AL amyloidosis and 7 cases (11.7%) of ATTR amyloidosis.

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来源期刊

Kardiologiya 医学-心血管系统

CiteScore

1.70

自引率

20.00%

发文量

审稿时长

3-8 weeks

期刊介绍： “Kardiologiya” (Cardiology) is a monthly scientific, peer-reviewed journal committed to both basic cardiovascular medicine and practical aspects of cardiology. As the leader in its field, “Kardiologiya” provides original coverage of recent progress in cardiovascular medicine. We publish state-of-the-art articles integrating clinical and research activities in the fields of basic cardiovascular science and clinical cardiology, with a focus on emerging issues in cardiovascular disease. Our target audience spans a diversity of health care professionals and medical researchers working in cardiovascular medicine and related fields. The principal language of the Journal is Russian, an additional language – English (title, authors’ information, abstract, keywords). “Kardiologiya” is a peer-reviewed scientific journal. All articles are reviewed by scientists, who gained high international prestige in cardiovascular science and clinical cardiology. The Journal is currently cited and indexed in major Abstracting & Indexing databases: Web of Science, Medline and Scopus. The Journal''s primary objectives Contribute to raising the professional level of medical researchers, physicians and academic teachers. Present the results of current research and clinical observations, explore the effectiveness of drug and non-drug treatments of heart disease, inform about new diagnostic techniques; discuss current trends and new advancements in clinical cardiology, contribute to continuing medical education, inform readers about results of Russian and international scientific forums; Further improve the general quality of reviewing and editing of manuscripts submitted for publication; Provide the widest possible dissemination of the published articles, among the global scientific community; Extend distribution and indexing of scientific publications in major Abstracting & Indexing databases.