统一谜题:全身水肿综合征谱系中的心脏淀粉样变性

Zahri Soukaina
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摘要

导言:针对肾病综合征和充血性心力衰竭带来的诊断挑战,本文强调了全面检查在揭示潜在病因方面的关键作用。在各种潜在病因中,淀粉样变性是一个独特的类别,其特征是淀粉样纤维在器官中的异常堆积。尽管淀粉样变性并不常见,但其严重性和潜在的针对性干预措施突出了在鉴别诊断中考虑淀粉样变性的重要性。病例介绍:对一名患有全身水肿综合征的 68 岁女性进行的详细病例研究显示,其临床表现复杂,涉及心脏和肾脏表现。通过全面的诊断评估,确诊为 AL 淀粉样变性,并指导采取了有针对性的治疗方法。讨论:讨论深入探讨了 AL 淀粉样变性的病理生理学、诊断注意事项以及心电图和超声心动图检查结果的重要性。讨论强调了实验室评估(尤其是免疫学检测)的重要性,并强调了活检在确诊中的作用。结论强调了 AL 淀粉样变性的复杂性,强调了心脏、肾脏和血液学方面的相互作用。早期诊断和量身定制的多学科治疗方法取得了积极的成果,为不断发展的淀粉样变性管理认识提供了宝贵的见解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unifying the Puzzle: Cardiac Amyloidosis in the Spectrum of Generalized Edematous Syndromes
Introduction: Addressing the diagnostic challenges posed by nephrotic syndrome and congestive heart failure, this article underscores the pivotal role of comprehensive investigations in unraveling underlying etiologies. Amid the diverse spectrum of potential causes, the focus is directed towards amyloidosis—a distinct category characterized by abnormal amyloid fibril accumulation in organs. Despite its infrequency, the gravity and potential for targeted interventions accentuate the importance of considering amyloidosis in the differential diagnosis. Case Presentation: A detailed case study of a 68-year-old female with generalized edematous syndrome revealed a complex clinical scenario involving cardiac and renal manifestations. AL amyloidosis was confirmed through thorough diagnostic assessments, guiding a targeted therapeutic approach. Discussion: The discussion delves into the pathophysiology of AL amyloidosis, diagnostic considerations, and the significance of ECG and echocardiographic findings. It highlights the importance of laboratory assessments, especially immunologic tests, and emphasizes the role of biopsy in confirming the diagnosis. Conclusion: The complexity of AL amyloidosis is underscored, emphasizing the interplay of cardiac, renal, and hematologic aspects. Early diagnosis and a tailored multidisciplinary therapeutic approach yielded positive outcomes, contributing valuable insights to the evolving understanding of amyloidosis management.
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