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一名成人斯蒂尔病患者的播散性脑血栓性微血管病

Disseminated cerebral thrombotic microangiopathy in a patient with adult's still disease.

作者信息

Domingues Renan B, da Gama Ana Maria Casati, Caser Eliana B, Musso Carlos, Santos Maria Carmen S

机构信息

Escola de Medicina, Santa Casa de Misericórdia de Vitória, Vitória ES, Brasil.

出版信息

Arq Neuropsiquiatr. 2003 Jun;61(2A):259-61. doi: 10.1590/s0004-282x2003000200018. Epub 2003 Jun 9.

Abstract

OBJECTIVE

The aim of this report is to describe a fatal disseminated thrombotic micoangiopathy with renal, pancreatic, and cerebral involvement in a patient with recently diagnosed adult's Still disease (ASD).

CASE REPORT

A previously healthy 15 year old girl was admitted to our hospital. According to the clinical and laboratory data the diagnosis of adult's still Disease was established. The treatment was begun and few days after an initial improvement a sudden neurologic deterioration with coma and seizures has occurred. Hours later the patient died. Clinical, laboratorial, and pathologic data will be presented.

CONCLUSION

To our knowledge this is the second description of a fatal disseminated cerebral thrombotic microangiopathy in a patient with adult's Still disease, but with a much more fulminating evolution than previously reported. Some etiopathogenic mechanisms could be shared in these two disorders explaining this coexistence.

摘要

目的

本报告旨在描述一例近期诊断为成人斯蒂尔病(ASD)的患者发生的致命性播散性血栓性微血管病,累及肾脏、胰腺和脑部。

病例报告

一名既往健康的15岁女孩入住我院。根据临床和实验室数据确诊为成人斯蒂尔病。开始治疗,在最初病情改善几天后,突然出现神经功能恶化,伴有昏迷和癫痫发作。数小时后患者死亡。将展示临床、实验室和病理数据。

结论

据我们所知,这是第二例关于成人斯蒂尔病患者发生致命性播散性脑血栓性微血管病的描述,但病情进展比之前报道的更为迅猛。这两种疾病可能存在一些共同的病因发病机制来解释这种共存现象。

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