Amyloid-Journal of Protein Folding Disorders最新文献_第6页

Identification of epidermal growth factor-containing fibulin-like extracellular matrix protein 1-derived amyloid deposition in a rhesus macaque. 在猕猴体内发现含表皮生长因子的纤维蛋白样细胞外基质蛋白 1 淀粉样沉积。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-10-29 DOI: 10.1080/13506129.2024.2421434

Matthew F Starost, Tomoaki Murakami, Kelli L Vaughan, Christopher King, Anna Harima, Julie A Mattison

引用次数: 0

Left atrioventricular coupling index assessed with three-dimensional echocardiography: a prognostic marker of short-term outcomes in light-chain cardiac amyloidosis. 三维超声心动图评估左房室耦合指数：轻链心脏淀粉样变性短期预后的预后指标。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2025-01-15 DOI: 10.1080/13506129.2024.2448435

Fangmin Meng, Jing Li, Rui Zhao, Yuanfeng Wu, Yu Liu, Yiming Yang, Yang Yang, Nianwei Zhou, Lili Dong, Dehong Kong, Haiyan Chen, Xianhong Shu, Peng Liu, Cuizhen Pan

{"title":"Left atrioventricular coupling index assessed with three-dimensional echocardiography: a prognostic marker of short-term outcomes in light-chain cardiac amyloidosis.","authors":"Fangmin Meng, Jing Li, Rui Zhao, Yuanfeng Wu, Yu Liu, Yiming Yang, Yang Yang, Nianwei Zhou, Lili Dong, Dehong Kong, Haiyan Chen, Xianhong Shu, Peng Liu, Cuizhen Pan","doi":"10.1080/13506129.2024.2448435","DOIUrl":"10.1080/13506129.2024.2448435","url":null,"abstract":"Background: Light-chain cardiac amyloidosis (AL-CA) is associated with structural and functional changes in the left atrium and left ventricle. This study aims to assess the value of the left atrioventricular coupling index (LACI) assessed by three-dimensional echocardiography (3DE) for predicting primary outcome in AL-CA.Methods: Participants with biopsy-confirmed AL-CA from April 2022 to February 2024 were prospectively analysed. LACI, the ratio of left atrial volume min to left ventricular end-diastolic volumes, was calculated offline using EchoPAC 204. The primary outcome was defined as all-cause death.Results: Sixty-seven biopsy-proven AL-CA patients were studied (age: 62.98 ± 10.20 years; 67% male). The median follow-up was 121 days (range: 7 ∼ 732 days). All-cause mortality occurred in 26 (39%) patients. Multivariate Cox regression revealed a significant association of LACI after adjusting for NT-pro BNP, troponin T, moderate tricuspid regurgitation, pericardial and pleural effusion (adjusted HR: 10.58, p = 0.008). Kaplan-Meier curves displayed prognostic differences based on median LACI (cut-off : 0.57, p = 0.002). The likelihood ratio χ2 test showed that LACI added predictive value to Mayo 2004, European 2015 modification of Mayo 2004, and Mayo 2012 models (All p < 0.001).Conclusions: 3DE-based LACI is independently associated with all-cause mortality in AL-CA patients and augments prognostic value to traditional staging models.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"63-71"},"PeriodicalIF":5.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143015615","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 impact of beta-blocker withdrawal in transthyretin amyloid cardiomyopathy. 经甲状腺素淀粉样变性心肌病患者停用β-受体阻滞剂的临床影响。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-11-20 DOI: 10.1080/13506129.2024.2431082

Eva Cabrera-Romero, Leidy Alexandra Serrao-Faria, Nerea Mora-Ayestarán, Belén Peiró-Aventín, Ana Espinoza, Daniel de Castro, Marta Cobo-Marcos, Fernando Domínguez, Esther González-López, Pablo Garcia-Pavia

引用次数: 0

Involvement of bile acid in diarrhoea and therapeutic effect of colestimide in hereditary ATTR amyloidosis. 胆汁酸对遗传性 ATTR 淀粉样变性病腹泻的影响及可乐定的治疗效果。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-11-20 DOI: 10.1080/13506129.2024.2430554

Yusuke Mochizuki, Nagaaki Katoh, Akira Matsushima, Masahide Yazaki, Naoko Kuwabara, Saori Nakagawa, Yoshiki Sekijima

{"title":"Involvement of bile acid in diarrhoea and therapeutic effect of colestimide in hereditary ATTR amyloidosis.","authors":"Yusuke Mochizuki, Nagaaki Katoh, Akira Matsushima, Masahide Yazaki, Naoko Kuwabara, Saori Nakagawa, Yoshiki Sekijima","doi":"10.1080/13506129.2024.2430554","DOIUrl":"10.1080/13506129.2024.2430554","url":null,"abstract":"Background: Diarrhoea is one of the most serious complications in hereditary ATTR (ATTRv) amyloidosis. However, its precise pathomechanism remains unknown. The present study investigated the involvement of bile acid in diarrhoea along with the therapeutic effect of colestimide, a bile acid sequestrant, in ATTRv amyloidosis.Methods: We prospectively enrolled 19 ATTRv amyloidosis patients (9 with refractory diarrhoea and 10 without diarrhoea) and 20 healthy individuals for measurements of serum 7a-hydroxy-4-cholesten-3-one (C4) levels. The patients with diarrhoea were then treated with oral colestimide (1.5 g twice daily) for 28 days. The frequency of diarrhoea and C4 level were evaluated before and after colestimide treatment.Results: Mean serum C4 level was significantly higher in ATTRv patients with diarrhoea (62.3 ng/mL) than in ATTRv patients without diarrhoea (24.0 ng/mL, p = 0.03). Colestimide treatment significantly decreased mean diarrhoea frequency (pre-treatment period: 9.1 times/week, colestimide treatment period, 6.6 times/week, p = 0.04) and increased mean C4 level (before treatment: 66.2 ng/mL, after treatment: 187.1 ng/mL, p = 0.02).Conclusions: Bile acid status was significantly associated with diarrhoea in ATTRv amyloidosis. Colestimide and other bile acid sequestrants may reduce diarrhoea frequency in afflicted patients.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"39-45"},"PeriodicalIF":5.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142677439","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

Right ventricular coupling predicts cardiopulmonary fitness in cardiac transthyretin amyloidosis. 右心室耦合预测心脏转甲状腺蛋白淀粉样变性患者的心肺健康。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-12-23 DOI: 10.1080/13506129.2024.2442994

Katharina Knoll, Stefan Gross, Patrick Fuchs, Amadea Erben, Julia Hock, Moritz von Scheidt, Thorsten Kessler, Carsten Lennerz, Wibke Reinhard, Heribert Schunkert, Teresa Trenkwalder

{"title":"Right ventricular coupling predicts cardiopulmonary fitness in cardiac transthyretin amyloidosis.","authors":"Katharina Knoll, Stefan Gross, Patrick Fuchs, Amadea Erben, Julia Hock, Moritz von Scheidt, Thorsten Kessler, Carsten Lennerz, Wibke Reinhard, Heribert Schunkert, Teresa Trenkwalder","doi":"10.1080/13506129.2024.2442994","DOIUrl":"10.1080/13506129.2024.2442994","url":null,"abstract":"Background: Wild-type transthyretin cardiac amyloidosis (ATTRwt) is an infiltrative disease leading to restrictive cardiomyopathy. We aimed to characterise exercise capacity in ATTRwt and to identify predictors of cardiopulmonary fitness, focusing on echocardiographic and clinical parameters.Methods: We studied 110 ATTRwt patients from a prospective single-centre registry (2020-2024) by cardiopulmonary exercise testing (CPET). Besides CPET, all patients underwent comprehensive clinical assessment including follow-up for mortality. In 32 patients follow-up CPET after 1 year was available.Results: In ATTRwt, reduced aerobic capacity (pVO2 16 [13-18] ml/kg/min), and ventilatory inefficiency (VE/VCO2 slope 35 [30-43]) were common. In the multivariable regression analysis, we identified TAPSE/sPAP ratio as predictive for pVO2 (p = 0.019) and ventilatory efficiency (p = 0.004), while left ventricular ejection fraction or measures of left ventricular hypertrophy were not predictive. Concordantly, TAPSE/sPAP ratio assessed at baseline predicted pVO2 at 1-year follow-up (p = 0.009). Furthermore, patients with a TAPSE/sPAP ratio below the median of 0.38 mm/mmHg presented a higher risk of all-cause death (p = 0.009).Conclusion: In ATTRwt the TAPSE/sPAP ratio, a marker of right ventricular coupling, was an independent predictor of aerobic capacity assessed by CPET, at baseline and after 1 year, highlighting the importance of right ventricular assessment for risk stratification.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"54-62"},"PeriodicalIF":5.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142878536","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

High frequency of occult transthyretin and apolipoprotein AI-type amyloid in aortic valves removed by valve replacement for aortic stenosis. 在因主动脉瓣狭窄而进行瓣膜置换术切除的主动脉瓣中，隐匿性转甲状腺素和脂蛋白AI型淀粉样蛋白的出现频率很高。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-11-11 DOI: 10.1080/13506129.2024.2426508

Kohei Honda, Masayoshi Tasaki, Tetsuhiro Yamano, Mitsuharu Ueda, Hironobu Naiki, Noriyuki Tanaka, Yukiko Morinaga, Aya Miyagawa-Hayashino

{"title":"High frequency of occult transthyretin and apolipoprotein AI-type amyloid in aortic valves removed by valve replacement for aortic stenosis.","authors":"Kohei Honda, Masayoshi Tasaki, Tetsuhiro Yamano, Mitsuharu Ueda, Hironobu Naiki, Noriyuki Tanaka, Yukiko Morinaga, Aya Miyagawa-Hayashino","doi":"10.1080/13506129.2024.2426508","DOIUrl":"10.1080/13506129.2024.2426508","url":null,"abstract":"Background: A high incidence of valvular involvement of amyloid in the setting of aortic stenosis (AS) has been reported. Amyloid derived from ApoAI (AApoAI) can form local amyloid deposits in the aortic valve. Although a high prevalence of concomitant severe AS and cardiac transthyretin-type amyloidosis (ATTR) has been reported, the prevalence of valvular involvement by ATTR and AApoAI is unclear.Methods: Using immunostaining and mass spectrometry, we analysed amyloid proteins in 97 aortic valves removed for valve replacement due to AS at Kyoto Prefectural University of Medicine between 2014 and 2021. Clinical information was also reviewed.Results: Amyloid deposits were found in 44 cases (45%), of which 30 cases (68%) involved ATTR and 33 cases (75%) AApoAI. Statistical analysis showed significantly lower age and E/e' among amyloid-positive cases compared with amyloid-negative cases and significantly lower brain natriuretic peptide, higher fractional shortening, and higher left ventricular ejection fraction among ATTR-positive cases compared with ATTR-negative cases. Seven recent patients underwent bone scintigraphy and ATTR cardiomyopathy was observed in only one case.Conclusions: AS symptoms can manifest earlier in patients with amyloid or ATTR deposition in the aortic valve than in patients without such deposition, even though left ventricular function is preserved.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"22-28"},"PeriodicalIF":5.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142631826","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

In memoriam: Lawreen Connors, Ph.D. 纪念：劳琳·康纳斯博士

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-03-01 Epub Date: 2024-11-27 DOI: 10.1080/13506129.2024.2432979

Vaishali Sanchorawala, Martha Skinner

引用次数: 0

Altered connectivity of central autonomic network: effects of dysautonomia in hereditary transthyretin amyloidosis with polyneuropathy. 中枢自律神经网络连接的改变：遗传性转甲状腺素淀粉样变性伴有多发性神经病的自律神经失调症的影响。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-12-01 Epub Date: 2024-07-24 DOI: 10.1080/13506129.2024.2383450

Tsai-Jou Su, Chien-Ho Janice Lin, Yen-Lin Liu, Hsueh-Wen Hsueh, Sung-Tsang Hsieh, Chi-Chao Chao, Ming-Chang Chiang

{"title":"Altered connectivity of central autonomic network: effects of dysautonomia in hereditary transthyretin amyloidosis with polyneuropathy.","authors":"Tsai-Jou Su, Chien-Ho Janice Lin, Yen-Lin Liu, Hsueh-Wen Hsueh, Sung-Tsang Hsieh, Chi-Chao Chao, Ming-Chang Chiang","doi":"10.1080/13506129.2024.2383450","DOIUrl":"10.1080/13506129.2024.2383450","url":null,"abstract":"Background: Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is a progressive fatal disorder caused by deposition of mutant transthyretin (TTR) amyloids mainly in the nerves and heart. Autonomic dysfunction is a major disabling manifestation, affecting 90% of patients with late-onset ATTRv-PN. The current study aimed to investigate brain functional alterations associated with dysautonomia due to peripheral autonomic nerve degeneration in ATTRv-PN.Methods: Resting-state functional MRI data were acquired from 43 ATTRv-PN patients predominantly of A97S (p.A117S) genotype, and the functional connectivity of central autonomic regions was assessed.Results: Compared with age-matched healthy controls, the ATTRv-PN patients exhibited (1) reduced functional connectivity of the central autonomic regions such as hypothalamus, amygdala, anterior insula, and middle cingulate cortex with brain areas of the limbic, frontal, and somatosensory systems, and (2) correlations of reduced functional autonomic connectivity with the severity of autonomic dysfunction especially orthostatic intolerance, decreased heart rate variability, and greater clinical disability.Conclusions: Our findings provide evidence linking peripheral autonomic dysfunction with altered connectivity in the central autonomic network in ATTRv-PN.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"257-265"},"PeriodicalIF":5.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141753334","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

Incidence and predictors of sudden death in patients with cardiac amyloidosis. 心脏淀粉样变性患者猝死的发生率和预测因素。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-12-01 Epub Date: 2024-10-24 DOI: 10.1080/13506129.2024.2414295

Fernando de Frutos, Giulia Saturi, Esther Gonzalez-Lopez, Maurizio Sguazzotti, Fernando Dominguez, Alberto Ponziani, Eva Cabrera-Romero, Angelo Giuseppe Caponetti, Sara Lozano, Paolo Massa, Belen Peiro-Aventin, Antonella Accietto, Nerea Mora-Ayestarán, Alessandro Giovannetti, Victor Castro-Urda, Christian Gagliardi, Marta Cobo-Marcos, Rafael Rios-Tamayo, Elena Biagini, Manuel Gomez-Bueno, Nazzareno Galiè, Javier Segovia-Cubero, Simone Longhi, Pablo Garcia-Pavia

{"title":"Incidence and predictors of sudden death in patients with cardiac amyloidosis.","authors":"Fernando de Frutos, Giulia Saturi, Esther Gonzalez-Lopez, Maurizio Sguazzotti, Fernando Dominguez, Alberto Ponziani, Eva Cabrera-Romero, Angelo Giuseppe Caponetti, Sara Lozano, Paolo Massa, Belen Peiro-Aventin, Antonella Accietto, Nerea Mora-Ayestarán, Alessandro Giovannetti, Victor Castro-Urda, Christian Gagliardi, Marta Cobo-Marcos, Rafael Rios-Tamayo, Elena Biagini, Manuel Gomez-Bueno, Nazzareno Galiè, Javier Segovia-Cubero, Simone Longhi, Pablo Garcia-Pavia","doi":"10.1080/13506129.2024.2414295","DOIUrl":"10.1080/13506129.2024.2414295","url":null,"abstract":"Introduction: Although sudden death (SD) is a recognized complication of cardiac amyloidosis, there is scarce data about its incidence, mechanisms, and predictors. The aim of this study was to describe incidence of SD and to analyze possible risk factors.Methods: Consecutive patients with ATTR or AL cardiac amyloidosis evaluated at two European centers were identified. SD was defined as unexpected death in clinically stable patients. Cox proportional hazard regression was performed to assess risk factors in univariate analysis. Those statistically significant were then assessed through age-adjusted multivariate analysis.Results: Analysis included 784 patients, 569 with ATTR amyloidosis (mean age 74.1 ± 12.1 years) and 215 with AL amyloidosis (mean age 64.5 ± 10.8 years). After a median follow-up of 1.9 years, SD rate at 2 years was 1.8% in ATTR. Previous pacemaker implantation (PPM) was associated with increased risk after age-adjusted analysis (HR 4.97; 95%CI: 1.39-17.7; p = 0.01). SD rate in AL amyloidosis patients at 2 years was 8.0% after a median follow-up of 1.2 years. Betablockers and NYHA III-IV were independently associated with an increased risk after age-adjusted multivariate analysis (HR 7.06 95%CI (2.31-21.5) p = 0.001) and (HR 4.56 95%CI (1.51-13.8) p = 0.007) respectively.Conclusions: SD is more frequent in AL than in ATTR cardiac amyloidosis. SD is associated with different risk factors in both entities.","PeriodicalId":50964,"journal":{"name":"Amyloid-Journal of Protein Folding Disorders","volume":" ","pages":"334-338"},"PeriodicalIF":5.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142512465","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 amyloidosis: the possibilities and challenges in the Ghanaian setting. 心脏淀粉样变性：加纳的可能性与挑战。

IF 5.2 2区医学

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-12-01 Epub Date: 2024-11-04 DOI: 10.1080/13506129.2024.2422458

Andrew Sefenu Dzebu, Magalys López Cuba

引用次数: 0