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Clinical profile and outcome of AA amyloidosis associated kidney disease in India. 印度AA淀粉样变性肾病的临床概况和预后
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-07 DOI: 10.1080/13506129.2025.2573233
Bhavik Bansal, Akash Sarkar, Adarsh Barwad, Geetika Singh, Arunkumar Subbiah, Raj Kanwar Yadav, Sandeep Mahajan, Dipankar Bhowmik, Sanjay Kumar Agarwal, Soumita Bagchi
{"title":"Clinical profile and outcome of AA amyloidosis associated kidney disease in India.","authors":"Bhavik Bansal, Akash Sarkar, Adarsh Barwad, Geetika Singh, Arunkumar Subbiah, Raj Kanwar Yadav, Sandeep Mahajan, Dipankar Bhowmik, Sanjay Kumar Agarwal, Soumita Bagchi","doi":"10.1080/13506129.2025.2573233","DOIUrl":"10.1080/13506129.2025.2573233","url":null,"abstract":"<p><strong>Background: </strong>AA amyloidosis is a rare but significant cause of chronic kidney disease (CKD). We aimed to characterize the clinical profile of patients with AA amyloidosis affecting the kidneys in the Indian subcontinent.</p><p><strong>Methodology: </strong>In this retrospective cohort study, we evaluated patients with kidney biopsy-confirmed AA amyloidosis and compared them with a control group of patients with diabetic kidney disease (DKD). Primary outcome was defined as a composite of ≥50% decline in estimated glomerular filtration rate (eGFR) and/or progression to end-stage kidney disease (ESKD).</p><p><strong>Results: </strong>AA amyloidosis (<i>n</i> = 91) accounted for 1.9% of all kidney biopsies. The median age was 45 years, and 75.8% were male. Chronic infections or inflammatory diseases were reported in 58.2%, tuberculosis being most common (35.2%). Baseline median eGFR was 66.0 mL/min/1.73 m<sup>2</sup> and urine protein creatinine ratio was 4.9 g/g. During median follow-up of 5.58 years, 38.6% experienced worsening kidney outcomes. Adjusted analyses showed significantly better kidney survival than DKD (0.29 (95% CI: 0.14-0.66, <i>p</i> = 0.002).</p><p><strong>Conclusion: </strong>AA amyloidosis is an uncommon but important cause of CKD. Tuberculosis is the leading predisposing factor in Indian patients. These patients exhibit slower eGFR decline compared to DKD despite progressive proteinuria, suggesting distinct pathophysiology.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"15-24"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145472422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Optimized methods for efficient application of immunogold electron microscopy to amyloid fibrils typing. 免疫金电镜对淀粉样蛋白原纤维分型的优化方法。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-23 DOI: 10.1080/13506129.2025.2589892
Yan Zhou, Weining Lu, Eric Burks, Joel M Henderson, Chris Andry, Vaishali Sanchorawala, Zahra Sheikh, Tatiana B Prokaeva, Hui A Chen
{"title":"Optimized methods for efficient application of immunogold electron microscopy to amyloid fibrils typing.","authors":"Yan Zhou, Weining Lu, Eric Burks, Joel M Henderson, Chris Andry, Vaishali Sanchorawala, Zahra Sheikh, Tatiana B Prokaeva, Hui A Chen","doi":"10.1080/13506129.2025.2589892","DOIUrl":"10.1080/13506129.2025.2589892","url":null,"abstract":"<p><strong>Objective: </strong>Amyloidosis is a group of disorders characterized by aggregation of abnormal amyloid protein in various tissues, often leading to organ dysfunction and failure. We optimized the immunogold electron microscopy (IEM) technique to enable efficient amyloid typing in 4% paraformaldehyde-fixed (PFA) and formalin-fixed paraffin-embedded (FFPE) tissues.</p><p><strong>Methods: </strong>The optimized IEM technique was applied to 151 Congo-red positive specimens from various tissues representing different amyloidosis types; 117 were fixed in 4% PFA and 34 were FFPE samples. Specimens were embedded in Lowicryl/K4M and stained with modified Richardson's blue solution to differentiate amyloid (lavender-plum coloration stained) from non-amyloid (blue stained) areas under light microscopy. Antibodies against kappa and lambda light chains, transthyretin and amyloid A were used for amyloid typing by IEM.</p><p><strong>Results: </strong>The optimized IEM technique enabled precise localization of amyloid deposits and rapid identification of target area under light microscopy for thin-section placement on nickel grids for immunogold staining. Of 151 specimens, 147 (97.4%) were classified as kappa or lambda light chains, transthyretin or amyloid A. Lambda light chain predominated in fat pad aspirates (56.8%), while transthyretin was most common in heart tissues (71.7%).</p><p><strong>Conclusion: </strong>This optimized IEM technique enhances the accuracy and efficiency of amyloid typing, especially in samples with trace amyloid deposit. It offers significant advantages over traditional epoxy embedding with toluidine blue staining, supporting timely clinical diagnosis and therapeutic decision-making.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"47-58"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145589781","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Limited diagnostic utility of systematic Congo red staining in bone marrow biopsies. 系统刚果红染色在骨髓活检中的诊断作用有限。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-27 DOI: 10.1080/13506129.2025.2593512
Jens Haugbølle Bjerre
{"title":"Limited diagnostic utility of systematic Congo red staining in bone marrow biopsies.","authors":"Jens Haugbølle Bjerre","doi":"10.1080/13506129.2025.2593512","DOIUrl":"10.1080/13506129.2025.2593512","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"98-100"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145642249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cardiac Phenotype and Clinical Outcomes in Randall Disease versus Light-Chain Amyloidosis. 兰德尔病与轻链淀粉样变性的心脏表型和临床结果。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-21 DOI: 10.1080/13506129.2025.2589888
Martin Nicol, Camille Cohen, Alexis Talbot, Mathilde Baudet, Stephanie Harel, Nathalie Forgeard, Bruno Royer, Floriane Theves, Tristan Vaugeois, Damien Logeart, Bertrand Arnulf
{"title":"Cardiac Phenotype and Clinical Outcomes in Randall Disease versus Light-Chain Amyloidosis.","authors":"Martin Nicol, Camille Cohen, Alexis Talbot, Mathilde Baudet, Stephanie Harel, Nathalie Forgeard, Bruno Royer, Floriane Theves, Tristan Vaugeois, Damien Logeart, Bertrand Arnulf","doi":"10.1080/13506129.2025.2589888","DOIUrl":"10.1080/13506129.2025.2589888","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"104-107"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145566210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical and radiological stabilization following vutrisiran treatment in transthyretin leptomeningeal amyloidosis: a case report. 经甲状腺素型瘦脑膜淀粉样变性的临床和放射学稳定：1例报告。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-10-29 DOI: 10.1080/13506129.2025.2579652
Simone Rossi, Luca Spinardi, Pietro Guaraldi, Andrea Mastrangelo, Mattia Riefolo, Alessandro Vaisfeld, Flavia Palombo, Simone Longhi, Rita Rinaldi, Alfredo Conti, Maria Pia Foschini, Maria Guarino
{"title":"Clinical and radiological stabilization following vutrisiran treatment in transthyretin leptomeningeal amyloidosis: a case report.","authors":"Simone Rossi, Luca Spinardi, Pietro Guaraldi, Andrea Mastrangelo, Mattia Riefolo, Alessandro Vaisfeld, Flavia Palombo, Simone Longhi, Rita Rinaldi, Alfredo Conti, Maria Pia Foschini, Maria Guarino","doi":"10.1080/13506129.2025.2579652","DOIUrl":"10.1080/13506129.2025.2579652","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"90-93"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145394962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prognostic significance of cardiac biomarkers in patients with AL amyloidosis treated with a daratumumab-based regimen. 心脏生物标志物在以daratumumab为基础的方案治疗AL淀粉样变性患者中的预后意义
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2026-01-06 DOI: 10.1080/13506129.2025.2611254
Taku Kikuchi, Ukyo Kondo, Shotaro Sugita, Miyu Watanabe, Chiaki Matsumoto, Moe Nomura-Yogo, Kodai Kunisada, Kota Sato, Tomomi Takei, Mizuki Ogura, Yu Abe, Osamu Hosoya, Tadao Ishida, Nobuhiro Tsukada
{"title":"Prognostic significance of cardiac biomarkers in patients with AL amyloidosis treated with a daratumumab-based regimen.","authors":"Taku Kikuchi, Ukyo Kondo, Shotaro Sugita, Miyu Watanabe, Chiaki Matsumoto, Moe Nomura-Yogo, Kodai Kunisada, Kota Sato, Tomomi Takei, Mizuki Ogura, Yu Abe, Osamu Hosoya, Tadao Ishida, Nobuhiro Tsukada","doi":"10.1080/13506129.2025.2611254","DOIUrl":"10.1080/13506129.2025.2611254","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"112-116"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145913783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Interactions between amyloid fibril proteins. 淀粉样纤维蛋白之间的相互作用。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-27 DOI: 10.1080/13506129.2025.2594130
Per Westermark
{"title":"Interactions between amyloid fibril proteins.","authors":"Per Westermark","doi":"10.1080/13506129.2025.2594130","DOIUrl":"10.1080/13506129.2025.2594130","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"1-2"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145642276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
An Accelerated Sonication-Assisted Preparation method for mass spectrometry-based identification of subtype specific amyloidogenic proteins in fat aspirates. 基于质谱法鉴定脂肪抽吸物中亚型特异性淀粉样蛋白的加速超声辅助制备方法。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-11-12 DOI: 10.1080/13506129.2025.2585277
Nicolai Bjødstrup Palstrøm, Caroline Alqvist Lindegaard, Aleksandra Rojek, Amanda Jessica Campbell, Charlotte Toftmann Hansen, Hanne E H Møller, Niels Abildgaard, Hans Christian Beck
{"title":"An Accelerated Sonication-Assisted Preparation method for mass spectrometry-based identification of subtype specific amyloidogenic proteins in fat aspirates.","authors":"Nicolai Bjødstrup Palstrøm, Caroline Alqvist Lindegaard, Aleksandra Rojek, Amanda Jessica Campbell, Charlotte Toftmann Hansen, Hanne E H Møller, Niels Abildgaard, Hans Christian Beck","doi":"10.1080/13506129.2025.2585277","DOIUrl":"10.1080/13506129.2025.2585277","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"94-97"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145497290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Failure to screen for monoclonal proteins in patients who undergo bone scintigraphy for suspected cardiac amyloidosis. 未能筛选单克隆蛋白的患者接受骨显像怀疑心脏淀粉样变性。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-10-15 DOI: 10.1080/13506129.2025.2573235
Adam Devine, Anja Selland, Jose Arriola-Montenegro, Valmiki Maharaj
{"title":"Failure to screen for monoclonal proteins in patients who undergo bone scintigraphy for suspected cardiac amyloidosis.","authors":"Adam Devine, Anja Selland, Jose Arriola-Montenegro, Valmiki Maharaj","doi":"10.1080/13506129.2025.2573235","DOIUrl":"10.1080/13506129.2025.2573235","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"82-84"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145294406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Tafamidis treatment and neuropsychiatric dysfunction in patients with cardiac aTTR: a post-hoc analysis of the ATTRACT trial. 他法非地治疗和心脏aTTR患者的神经精神功能障碍：对attraction试验的事后分析。
IF 7.4 2区 医学
Amyloid-Journal of Protein Folding Disorders Pub Date : 2026-03-01 Epub Date: 2025-10-15 DOI: 10.1080/13506129.2025.2564232
Lark Steafo, Ujala Zubair, Julie George, Joel Skaistis
{"title":"Tafamidis treatment and neuropsychiatric dysfunction in patients with cardiac aTTR: a post-hoc analysis of the ATTRACT trial.","authors":"Lark Steafo, Ujala Zubair, Julie George, Joel Skaistis","doi":"10.1080/13506129.2025.2564232","DOIUrl":"10.1080/13506129.2025.2564232","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"72-74"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145304183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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