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肿瘤肾脏病学实践中的淀粉样变性:一份基于多学科病例的会议报告。

Amyloidoses in Onco-Nephrology Practice: A Multidisciplinary Case-Based Conference Report.

作者信息

Kitchlu Abhijat, Chan Christopher T, Jhaveri Kenar D, Delgado Diego, Tam Paul

机构信息

Department of Medicine, Division of Nephrology, University Health Network, University of Toronto, ON, Canada.

The Glomerular Disease Center at Northwell Health, Division of Kidney Diseases and Hypertension, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA.

出版信息

Can J Kidney Health Dis. 2023 Apr 18;10:20543581231165711. doi: 10.1177/20543581231165711. eCollection 2023.

Abstract

INTRODUCTION AND OBJECTIVE

Amyloidoses are a heterogeneous group of disorders resulting from deposition of amyloid fibrils into extracellular tissues. While the kidneys are one of the most frequent sites of amyloid deposition, amyloid deposits can also affect a wide range of organ systems, including the heart, liver, gastrointestinal tract, and peripheral nerves. The prognosis of amyloidosis, especially with cardiac involvement, remains poor; however, a collaborative approach applying new tools for diagnosis and management may improve outcomes. In September 2021, the Canadian Onco-Nephrology Interest Group hosted a symposium to discuss diagnostic challenges and recent advances in the management of amyloidosis from the perspectives of the nephrologist, cardiologist, and onco-hematologist.

METHODS AND SOURCES OF INFORMATION

Through structured presentations, the group discussed a series of cases highlighting the varied clinical presentations of amyloidoses affecting the kidney and heart. Expert opinions, clinical trial findings, and publication summaries were used to illustrate patient-related and treatment-related considerations in the diagnosis and management of amyloidoses.

KEY FINDINGS

(1) Overview of the clinical presentation of amyloidoses and the role of specialists in performing timely and accurate diagnostic workup; (2) review of best practices for multidisciplinary management of amyloidosis, including prognostic variables and determinants of treatment response; and (3) update on new and emerging treatments in the management of light chain and amyloid transthyretin amyloidoses.

LIMITATIONS

This conference featured multidisciplinary discussion of cases, and learning points reflect the assessments by the involved experts/authors.

IMPLICATIONS

Identification and management of amyloidoses can be facilitated with a multidisciplinary approach and higher index of suspicion from cardiologists, nephrologists, and hemato-oncologists. Increased awareness of clinical presentations and diagnostic algorithms for amyloidosis subtyping will lead to more timely interventions and improved clinical outcomes.

摘要

引言与目的

淀粉样变性是一组异质性疾病,由淀粉样原纤维沉积于细胞外组织所致。肾脏是淀粉样蛋白沉积最常见的部位之一,但淀粉样沉积物也可累及广泛的器官系统,包括心脏、肝脏、胃肠道和周围神经。淀粉样变性的预后,尤其是合并心脏受累时,仍然很差;然而,采用新的诊断和管理工具的协作方法可能会改善预后。2021年9月,加拿大肿瘤肾脏病学兴趣小组举办了一次研讨会,从肾脏病学家、心脏病学家和肿瘤血液学家的角度讨论淀粉样变性诊断方面的挑战和管理方面的最新进展。

方法与信息来源

通过结构化报告,该小组讨论了一系列病例,突出了影响肾脏和心脏的淀粉样变性的不同临床表现。专家意见、临床试验结果和出版物摘要被用于阐述淀粉样变性诊断和管理中与患者及治疗相关的考虑因素。

主要发现

(1)淀粉样变性的临床表现概述以及专家在进行及时准确的诊断检查中的作用;(2)淀粉样变性多学科管理的最佳实践回顾,包括预后变量和治疗反应的决定因素;(3)轻链和转甲状腺素蛋白淀粉样变性管理中新兴治疗方法的最新情况。

局限性

本次会议以病例的多学科讨论为特色,学习要点反映了相关专家/作者的评估。

启示

多学科方法以及心脏病学家、肾脏病学家和血液肿瘤学家更高的怀疑指数有助于淀粉样变性的识别和管理。提高对淀粉样变性亚型临床表现和诊断算法的认识将带来更及时的干预并改善临床结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/25aa/10123889/b2a53afbddf5/10.1177_20543581231165711-fig1.jpg

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