Amyloid-Journal of Protein Folding Disorders最新文献

{"title":"Bile cast nephropathy presenting as acute kidney injury in systemic light chain amyloidosis with severe hepatic involvement.","authors":"Rodolfo Garza-Morales, Liying Fu, Julie L Rosenthal, Udit Yadav","doi":"10.1080/13506129.2025.2572588","DOIUrl":"10.1080/13506129.2025.2572588","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"101-103"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145369217","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}

{"title":"AA amyloidosis as an adverse event of immune checkpoint inhibitor therapy: evidence from the FDA adverse event reporting system and a systematic review.","authors":"Umur Topcu, Buğra Han Esen, Sevval Nur Bektas, Fatih Selçukbiricik, Mehmet Kanbay","doi":"10.1080/13506129.2025.2589197","DOIUrl":"10.1080/13506129.2025.2589197","url":null,"abstract":"<p><strong>Background: </strong>Immune checkpoint inhibitors (ICIs) have transformed cancer therapy but are linked to immune-related adverse events (irAEs). Secondary (AA) amyloidosis, an inflammatory complication involving serum amyloid A deposition, has been sporadically reported in ICI-treated patients. We investigated the link between ICI therapy and AA amyloidosis using pharmacovigilance data and a systematic review.</p><p><strong>Methods: </strong>We conducted a disproportionality analysis of FDA adverse event reporting system (FAERS) data (January 2015-June 2024), selecting amyloidosis-related cases using MedDRA preferred terms. Signal detection employed reporting odds ratio, proportional reporting ratio, information component, and empirical Bayesian geometric mean. We also reviewed published cases from six databases (searched 30 November 2024). Studies reporting AA amyloidosis linked to ICI use were included, and the risk of bias was assessed using the Joanna Briggs Institute tools. Results were summarised descriptively (PROSPERO ID: CRD42024622091).</p><p><strong>Results: </strong>Among 13,209,688 unique FAERS reports, 26 relevant cases were identified. Patients were mainly older adults (median age 71.5 years), with serious outcomes including death (19.2%) and hospitalisation (30.8%). Disproportionality analyses showed a significant link, and the systematic review included 11 cases, primarily renal, with poor outcomes despite treatment.</p><p><strong>Conclusion: </strong>Findings support AA amyloidosis as a rare but serious irAE of ICI therapy, warranting further investigation.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"25-35"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582770","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}

{"title":"A comparison of single versus combination mechanism treatment for transthyretin amyloid cardiomyopathy.","authors":"Vrinda Gupta, Lisa R Yanek, Bairavi Shankar, Artrish Jefferson, Daniel Tsottles, Serena Zampino, Jennifer Barranco, Muhammed Rahim, Emily Brown, Mark Ranek, Nisha Gilotra, Kavita Sharma, Michael Polydefkis, Joban Vaishnav","doi":"10.1080/13506129.2025.2570095","DOIUrl":"10.1080/13506129.2025.2570095","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"79-81"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145287645","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}

{"title":"Neuropathy impairment and nutritional status with eplontersen in patients with hereditary transthyretin-mediated amyloidosis.","authors":"Jonas Wixner, Isabel Conceição, John L Berk, David Adams, Michael J Polydefkis, Shahram Attarian, Julian D Gillmore, P James B Dyck, Jersey Chen, T Jesse Kwoh, Jonatan Nåtman, Wunan Zhou, Márcia Waddington Cruz","doi":"10.1080/13506129.2025.2577361","DOIUrl":"10.1080/13506129.2025.2577361","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"85-89"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145379770","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}

{"title":"The role of cardiac imaging for diagnosis of cardiac amyloidosis: a systematic review and meta-analysis of test accuracy.","authors":"Hassan Kawtharany, Muayad Azzam, Jamil Nazzal, Qais Hamarsha, Aseel Alkhader, Tala Khraise, Mohammad M AlMasri, Hadi K Abou Zeid, Iktimal Alwan, Mhd Amin Alzabibi, Nour Jaber, Faizi Jamal, Noel Dasgupta, Nitasha Sarswat, Alfredo H De La Torre, Maria Adela Aguirre, Deborah Boedicker, Naresh Bumma, Antonia S Carroll, Raymond Comenzo, Joselle Cook, Angela Dispenzieri, Jack Khouri, Maria M Picken, Shahzad Raza, Nelson Leung, Vaishali Sanchorawala, Hira Shaikh, Daulath Singh, Matthew D Seftel, Vishal Kukreti, Reem A Mustafa","doi":"10.1080/13506129.2025.2596145","DOIUrl":"10.1080/13506129.2025.2596145","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the diagnostic test accuracy of cardiac magnetic resonance (CMR) and echocardiography for diagnosis of cardiac involvement in patients with biopsy proven light-chain (AL) amyloidosis.</p><p><strong>Methods: </strong>This systematic review addresses late gadolinium enhancement (LGE) on CMR and different echocardiographic findings for cardiac involvement using an acceptable reference standard. Meta-analysis reported sensitivity and specificity with 95% confidence intervals when we have ≥3 studies, or ranges for 2 studies. We assessed certainty of evidence using GRADE (Grading of Recommendations Assessment, Development, and Evaluation).</p><p><strong>Results: </strong>Seven studies evaluated LGE, yielding pooled sensitivity and specificity of 0.95 (0.87-0.98) and 0.87 (0.76-0.94). Nineteen studies addressed echocardiography. Interventricular septum thickness showed sensitivity of 0.77 (0.69-0.84) and specificity of 0.71 (0.60-0.81). Diastolic dysfunction (grade 2-3) sensitivity was 0.71 (0.40-0.90) and specificity was 0.75 (0.64-0.84); restrictive filling pattern (grade 3) sensitivity was 0.42 (0.28-0.58) and specificity 0.89 (0.83-0.94). E/A ratio sensitivity ranged from 0.45 to 0.65, with specificity from 0.85 to 0.98. Global longitudinal strain sensitivity was 0.86 (0.65-0.95) and specificity was 0.76 (0.55-0.89). Apical sparing pattern showed sensitivity of 0.72 (0.64-0.78) and specificity of 0.78 (0.64-0.88). Certainty of evidence was very low.</p><p><strong>Conclusion: </strong>CMR might be more accurate than echocardiography for diagnosis of cardiac involvement in AL amyloidosis.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"59-71"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890481","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}

{"title":"Amyloid myopathy in the internal oblique muscle of patients with wild-type transthyretin cardiac amyloidosis.","authors":"Koji Takahashi, Takaaki Iwamura, Yoshiyasu Hiratsuka, Shuhei Yamamoto, Daisuke Sasaki, Sohei Kitazawa, Nobuhisa Yamamura, Mitsuharu Ueda, Hiroe Morioka, Shigeki Uemura, Tomoki Sakaue, Katsuji Inoue","doi":"10.1080/13506129.2025.2564897","DOIUrl":"10.1080/13506129.2025.2564897","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"75-78"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145276584","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}

{"title":"Medin and transthyretin: a new amyloid double act in the aortic wall and valves.","authors":"Alana Maerivoet, Rebecca Price, Riaz Akhtar, Mark Field, Jillian Madine","doi":"10.1080/13506129.2025.2554880","DOIUrl":"10.1080/13506129.2025.2554880","url":null,"abstract":"<p><strong>Background: </strong>Cross-seeding and co-assembly of multiple amyloid species are increasingly recognised in various organs and amyloidoses. Medin and wild-type transthyretin (TTR) both form age-related amyloid deposits and have been identified within the aortic wall. Given the emerging role of amyloid in aortic disease, this study investigates the potential colocalisation of TTR and medin in the aorta.</p><p><strong>Methods: </strong>Medin and TTR levels were measured in thoracic aortic wall samples from 30 patients undergoing surgical replacement for aortic aneurysm. Immunohistochemistry was performed on five aortic wall samples and two excised aortic valves to assess colocalisation patterns. <i>In vitro</i> assays, including Thioflavin T fluorescence and immunogold labelling electron microscopy, evaluated protein co-aggregation and fibril formation. Cellular toxicity assays examined the impact of TTR on medin aggregates.</p><p><strong>Results: </strong>A positive correlation was observed between medin and TTR levels in the aortic wall. <i>In vitro</i> assays revealed enhanced fibril formation and co-aggregation, with TTR reducing the cellular toxicity of medin aggregates, suggesting altered fibril properties. Immunohistochemistry confirmed colocalisation of medin and TTR in both aortic wall and valve samples.</p><p><strong>Conclusions: </strong>This study identifies a novel association between medin and TTR, highlighting a potential role for co-aggregation in vascular amyloid deposition.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"3-14"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144977584","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}

{"title":"Cold pressor test and paradoxical blood pressure reduction in light chain amyloidosis.","authors":"Raphael Patras, Georgios Georgiopoulos, Foteini Theodorakakou, Ioannis Petropoulos, Dimitris Delialis, Lasthenis Angelidakis, Alexandros Briasoulis, Maria Gavriatopoulou, Panagiotis Kokotis, Efstathios Manios, Meletios-Athanasios Dimopoulos, Efstathios Kastritis, Kimon Stamatelopoulos","doi":"10.1080/13506129.2025.2577797","DOIUrl":"10.1080/13506129.2025.2577797","url":null,"abstract":"<p><strong>Background: </strong>Patients with AL amyloidosis present sustained paradoxical vasodilation in response to sympathetic stimulation by cold pressor test (CPT). The clinical relevance is unknown.</p><p><strong>Methods: </strong>Peripheral and central systolic (SBP) and diastolic blood pressure (DBP) were measured at 3 min (end of CPT) and at 6 min, before and 12 months after treatment initiation in 113 treatment-naïve AL amyloidosis patients. Ten healthy volunteers were assessed at baseline. All-cause and cardiovascular mortality were recorded.</p><p><strong>Results: </strong>Percentage reductions in central SBP and peripheral DBP at 6 min [AL vs controls: %CSBP6min (mean, SD) 0.7 ± 8.44 vs 7.57 ± 5.59; %DBP6min (mean, SD) 0.64 ± 8.91 vs 14 ± 9.11; <i>p</i> < 0.01 for both] were associated with all-cause and cardiovascular death (%CSBP6min HR = 0.945 and HR = 0.946 respectively; %DBP6min HR = 0.949 and HR = 0.916 respectively; <i>p</i> < 0.05 for all) after adjustment for disease-related risk factors. %CSBP6min provided incremental value over Mayo stage and was associated with neurological and cardiac dysfunction and myocardial infiltration. At 12 months, %CSBP6min further decreased in patients with earlier poor hematologic treatment response.</p><p><strong>Conclusions: </strong>%CSBP6min, a noninvasive, readily available marker, was associated with cardiac dysfunction, poor survival, and its further post-treatment reduction was associated with hematologic response.</p>","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"36-46"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145369174","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}

{"title":"Frontline Dara-CyBorD for AL amyloidosis: high response rates and cytogenetic insights from a real-world cohort.","authors":"Xia Wu, Eugène Brailovski, Denis Toskic, Stephanie Scalia, Ping Zhou, Xun Ma, Vasil Mico, Teresa Fogaren, Nancy Coady Lyons, Heather Landau, Raymond Comenzo","doi":"10.1080/13506129.2025.2592628","DOIUrl":"10.1080/13506129.2025.2592628","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"108-111"},"PeriodicalIF":7.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145662672","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}

{"title":"In memoriam: Shukuro Araki, MD, PhD (1927-2025) pioneer of Japanese neurology and a global leader in familial amyloid polyneuropathy.","authors":"Mitsuharu Ueda, Yohei Misumi, Yukio Ando","doi":"10.1080/13506129.2026.2622971","DOIUrl":"https://doi.org/10.1080/13506129.2026.2622971","url":null,"abstract":"","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"1-2"},"PeriodicalIF":7.4,"publicationDate":"2026-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146203558","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}