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上肢钙化防御——一种需要多学科治疗的罕见且严重的疾病——病例系列及文献综述

Calciphylaxis of the Upper Limbs-A Rare and Serious Disease with Multidisciplinary Treatments-A Case Series and Literature Review.

作者信息

Pertea Mihaela, Benamor Malek, Bulgaru-Iliescu Andra-Irina, Abid Abderrazek, Abid Said, Amarandei Alexandru-Hristo

机构信息

Department Plastic Surgery and Reconstructive, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.

Department of Plastic Surgery and Reconstructive Microsurgery, "Sf. Spiridon" Emergency County Hospital, 700111 Iasi, Romania.

出版信息

Diagnostics (Basel). 2025 May 6;15(9):1179. doi: 10.3390/diagnostics15091179.

Abstract

Calciphylaxis or calcific uremic arteriolopathy is a rare but highly lethal pathology that occurs most frequently in a uremic context, although it can also occur outside of this context. It is characterized by the appearance of necrotic skin lesions. Localization to the upper limbs is rare and has a similarly progressive evolution. We present a series of two cases-a male and a female-with calciphylaxis diagnoses (including biopsies) and with the patients undergoing dialysis for end-stage renal disease, both with infected and extensive necrotic lesions to the hands and fingers. Both cases required serial debridement treatments and amputations. A literature review was conducted using the precise search terms "calciphylaxis", "upper limb", "uremic calcific arteriolopathy", and "end-stage renal disease" from January 2010 to May 2024. One of the two reported cases ended with the patient's death. The results of the literature review (comprising seven similar cases) confirmed the rarity of calciphylaxis lesion localization to the upper limbs and the high mortality rate among these patients despite administered treatments. No therapeutic protocol for these cases was confirmed. The treatment of calciphylaxis cases is multidisciplinary. Although surgical intervention is controversial, it is necessary in some cases, sometimes serially. Localization to the thoracic limbs has the same evolution and poor prognosis as other localizations. A standardized therapeutic protocol for these cases is still far from being established.

摘要

钙化防御或钙化性尿毒症小动脉病是一种罕见但致死率很高的病理状况,最常发生于尿毒症背景下,不过也可能在这种背景之外出现。其特征是出现坏死性皮肤病变。上肢受累情况罕见,且病情发展类似。我们报告了两例病例——一男一女——均诊断为钙化防御(包括活检),且均因终末期肾病接受透析治疗,两人手部和手指均有感染且广泛的坏死性病变。两例均需多次清创治疗及截肢。我们使用“钙化防御”“上肢”“尿毒症性钙化小动脉病”和“终末期肾病”等精确检索词,对2010年1月至2024年5月的文献进行了综述。两例报告病例中有一例以患者死亡告终。文献综述结果(包括七例类似病例)证实了钙化防御病变累及上肢情况罕见,且尽管进行了治疗,这些患者的死亡率仍很高。未证实针对这些病例的治疗方案。钙化防御病例的治疗是多学科的。尽管手术干预存在争议,但在某些情况下是必要的,有时需要多次进行。上肢受累与其他部位受累的病情发展及预后相同。针对这些病例的标准化治疗方案仍远未确立。

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