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重症温德利希综合征的管理:一例病例报告及治疗策略综述

Management of Critical Wunderlich Syndrome: A Case Report and Review of Therapeutic Strategies.

作者信息

Danaf Samer, Tlaiss Yehya, Chams Mohamad, Assaf Georges, Moussa Mohammad, Ghantous Imad

机构信息

Department of Urology, University of Balamand, Beirut, Lebanon.

University of Balamand, Beirut, Lebanon.

出版信息

Case Rep Urol. 2025 Mar 15;2025:2129870. doi: 10.1155/criu/2129870. eCollection 2025.

DOI:10.1155/criu/2129870
PMID:40124902
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11929593/
Abstract

This case report presents a compelling instance of Wunderlich syndrome (WS), a rare and nontraumatic medical condition characterized by spontaneous renal hemorrhage into the subcapsular and perirenal spaces. WS poses unique diagnostic and management challenges due to its sudden and substantial hemorrhage presentation that is life-threatening, often accompanied by acute flank pain, hemodynamic instability, and the presence of a flank mass. While the exact pathogenesis remains debated, WS can arise from various renal pathologies, including neoplastic and nonneoplastic conditions. In this case, a 65-year-old male with a complex medical history, including polycystic kidney disease, presented to the emergency room with massive hematuria leading to an abrupt severe drop in hemoglobin levels and hemodynamic instability. Despite aggressive management, including transfusions, the patient ultimately underwent an urgent open left radical nephrectomy due to the severity of the condition and distorted anatomy. Additionally, the report draws attention to the potential use of tranexamic acid in WS cases, stressing the importance of balancing its benefits against associated risks. This case highlights the critical importance of recognizing and promptly addressing WS, highlighting the diverse etiology of this condition, the role of tranexamic acid in controlling bleeding, and the lifesaving role of nephrectomy in cases of significant bleeding and hemodynamic instability.

摘要

本病例报告展示了温德利希综合征(WS)的一个典型案例,这是一种罕见的非创伤性疾病,其特征为肾脏自发性出血进入肾包膜下和肾周间隙。WS因其突然且大量的出血表现(危及生命,常伴有急性胁腹痛、血流动力学不稳定以及胁腹肿块)而带来独特的诊断和管理挑战。虽然确切的发病机制仍存在争议,但WS可由多种肾脏病变引起,包括肿瘤性和非肿瘤性疾病。在此病例中,一名65岁男性,有包括多囊肾病在内的复杂病史,因大量血尿导致血红蛋白水平急剧严重下降和血流动力学不稳定而被送往急诊室。尽管采取了积极的治疗措施,包括输血,但由于病情严重且解剖结构紊乱,患者最终接受了紧急开放性左肾根治性切除术。此外,该报告还提请注意氨甲环酸在WS病例中的潜在用途,强调在其益处与相关风险之间进行权衡的重要性。本病例突出了识别并及时处理WS的至关重要性,凸显了该疾病的多种病因、氨甲环酸在控制出血中的作用以及肾切除术在严重出血和血流动力学不稳定病例中的救命作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a7/11929593/e45c9868d168/CRIU2025-2129870.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a7/11929593/e45c9868d168/CRIU2025-2129870.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a7/11929593/e45c9868d168/CRIU2025-2129870.001.jpg

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本文引用的文献

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A Case Report of Wünderlich Syndrome Causing Massive Hemorrhage During Hemodialysis.一例导致血液透析期间大出血的温德利希综合征病例报告。
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Spontaneous rupture of a giant renal angiomyolipoma-Wunderlich's syndrome: Report of a case.巨大肾血管平滑肌脂肪瘤自发破裂——温德利希综合征:病例报告
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