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一名七岁女孩患过敏性紫癜并发后部可逆性脑病综合征。

Posterior reversible encephalopathy syndrome as a complication of Henoch-Schönlein purpura in a seven-year-old girl.

作者信息

Dos Santos Daiane, Langer Felipe Welter, Dos Santos Tatiane, Rafael Tronco Alves Giordano, Feiten Marisa, Teixeira de Paula Neto Walter

机构信息

1 Department of Radiology and Imaging Diagnosis, University Hospital of Santa Maria, Federal University of Santa Maria, Brazil.

2 Luteran University of Brazil, Canoas, Brazil.

出版信息

Scott Med J. 2017 Feb;62(1):34-37. doi: 10.1177/0036933017690467. Epub 2017 Feb 2.

Abstract

Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

摘要

引言

过敏性紫癜是一种多系统小血管炎。神经系统表现并不常见。后部可逆性脑病综合征是过敏性紫癜的一种罕见并发症,具有典型的临床和神经影像学表现,最常发生在严重高血压和肾损伤的情况下。

病例报告

一名七岁女孩因临床和实验室检查结果提示过敏性紫癜入住我院。开始使用糖皮质激素治疗,但入院五天后,她出现意识改变、癫痫发作、动脉高血压和皮质盲。脑部磁共振成像扫描显示顶枕叶有血管源性水肿区域,符合后部可逆性脑病综合征。她立即开始使用抗高血压药和抗癫痫药,这些药物成功改善了她的神经症状。进一步的实验室检查发现继发于过敏性紫癜的快速进行性肾小球肾炎,这可能是她血压突然升高的原因。进行了免疫抑制治疗,在一年的随访中,患者的肾脏和神经功能完全恢复。

结论

后部可逆性脑病综合征是过敏性紫癜的一种严重并发症。如果及时诊断和治疗,患有后部可逆性脑病综合征的过敏性紫癜患儿通常预后良好。临床医生应熟悉后部可逆性脑病综合征的特征性表现,并意识到高血压和肾损伤可能使过敏性紫癜患者易发生这种并发症。

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