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终末期肾病患者的晚期钙化防御:一例报告,凸显晚期表现中的诊断和治疗挑战

Advanced Calciphylaxis in a Patient With End-Stage Renal Disease: A Case Report Highlighting Diagnostic and Therapeutic Challenges in Late-Stage Presentation.

作者信息

Chaganlal Priam P, Kalandoor Varun, Jones Daniel T, Pace Christopher, Silver Scott A

机构信息

Internal Medicine, Touro University Nevada, Henderson, USA.

Internal Medicine, Valley Hospital Medical Center, Las Vegas, USA.

出版信息

Cureus. 2024 Sep 7;16(9):e68866. doi: 10.7759/cureus.68866. eCollection 2024 Sep.

Abstract

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rapidly progressive, rare, and severe condition characterized by vascular calcification and skin necrosis. The pathophysiology involves cutaneous arteriolar calcification followed by subsequent tissue ischemia and infarction, which eventually causes extremely painful skin lesions. The condition is associated with substantial morbidity due to severe pain, non-healing wounds, increased susceptibility to infections, and frequent hospitalizations. Calciphylaxis is a highly fatal condition with one-year mortality rates greater than 50%, most frequently due to sepsis. This report presents a case of a 63-year-old male with end-stage kidney disease (ESKD) who presented with altered mental status and was found to have notable necrotic skin ulcers on the bilateral anterior thighs, a stage IV sacral decubitus ulcer, and necrotic lesions on the scrotum and penis. This case underscores the importance of maintaining a high clinical suspicion for rare conditions like calciphylaxis in patients with multiple risk factors. Diagnosing the disease earlier in its course may improve outcomes and overall prognosis. Unfortunately, in this case, the patient presented too late into the disease course, and ultimately discussions/placement with palliative care were undertaken.

摘要

钙化防御,也称为钙化性尿毒症小动脉病,是一种快速进展、罕见且严重的疾病,其特征为血管钙化和皮肤坏死。病理生理学过程包括皮肤小动脉钙化,随后是组织缺血和梗死,最终导致极其疼痛的皮肤病变。由于严重疼痛、伤口不愈合、感染易感性增加以及频繁住院,该疾病会导致相当高的发病率。钙化防御是一种高致死性疾病,一年死亡率超过50%,最常见的死因是败血症。本报告介绍了一例63岁终末期肾病(ESKD)男性患者,该患者出现精神状态改变,双侧大腿前部有明显的坏死性皮肤溃疡、IV期骶部压疮以及阴囊和阴茎的坏死性病变。该病例强调了对具有多种危险因素的患者保持高度临床怀疑以诊断像钙化防御这样罕见疾病的重要性。在病程早期诊断该疾病可能会改善预后和总体转归。不幸的是,在本病例中,患者就诊时疾病已进展到晚期,最终进行了姑息治疗的讨论/安排。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d32/11380459/7618f66101d0/cureus-0016-00000068866-i01.jpg

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