淀粉样轻链淀粉样变性并发急性心力衰竭和肾衰竭。

Kyle Cass, Lila Luna, William Kivlin, Buckley Fechter, Hari Paudel
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引用次数: 0

摘要

简介:淀粉样轻链(AL)淀粉样变性是一种多系统疾病,患者的表现差异很大。本病例描述了一名初次就诊时独特的多器官受累患者的表现和检查情况:病例介绍:一名 69 岁的非裔美国男性患者因虚弱、腿部肿胀和呼吸急促而就诊。初步检查显示患者患有急性心力衰竭和急性慢性肾功能衰竭,并伴有肾病范围蛋白尿(蛋白与肌酐比值为 5.78)。进一步检查显示,血清蛋白电泳、尿蛋白电泳和轻链均升高。随后的肾活检显示出λ受限的AL型肾淀粉样变性:讨论:文献中描述了多种系统性表现,但以心衰和肾衰并发为主要表现的病例并不多见:本病例强调了在对不明原因的多器官疾病患者进行鉴别诊断时考虑淀粉样变性等全身性炎症性疾病的重要性。早期诊断和治疗对于改善患者预后至关重要。提高对常见临床表现和实验室异常的识别能力将有可能通过早期诊断改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Concurrent Acute Heart Failure and Renal Failure in Amyloid Light Chain Amyloidosis.

Introduction: Amyloid light chain (AL) amyloidosis is a multisystem disease with significant variability in patient presentation. This case describes the presentation and workup of a patient with unique multiorgan involvement on initial presentation.

Case presentation: A 69-year-old African American male presented with weakness, leg swelling, and shortness of breath. Initial workup demonstrated acute heart failure and acute-on-chronic renal failure with nephrotic range proteinuria (5.78 protein to creatinine ratio). Further workup showed elevated serum protein electrophoresis, urine protein electrophoresis, and light chains. Subsequent renal biopsy showed lambda-restricted AL-type renal amyloidosis.

Discussion: A variety of systemic presentations have been described in the literature; however, concurrent heart and renal failure as primary presentation is uncommon.

Conclusions: This case emphasizes the importance of considering systemic inflammatory diseases, such as amyloidosis, in the differential diagnoses of patients with unexplained multiorgan disease. Early diagnosis and treatment initiation are essential for improving patient outcomes. Improved recognition of common clinical manifestations and laboratory abnormalities will likely improve outcomes through earlier diagnosis.

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