[后可逆性脑病综合征(PRES)和溶血性贫血:链球菌感染后急性肾小球肾炎的两种严重而不寻常的并发症]。

Gianfranco Tomarelli R, Daniela Arriagada S, Alejandro Donoso F
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引用次数: 0

摘要

小儿肾炎综合征最常见的原因是急性感染后肾小球肾炎(PIGN)。后部可逆性脑病综合征(PRES)是一种罕见的并发症,其特征是皮层下血管源性脑水肿伴各种神经系统症状。自身免疫性溶血性贫血是一种不典型的临床表现。目的:报道两种罕见且严重的PIGN外并发症的共存,并探讨其发展的可能机制。临床病例:男性,4岁,血尿、水肿、头痛、恶心、呕吐5天。患者入院时处于惊厥状态和高血压危象,实验室显示C3低补体血症和抗溶血素O高滴度,解释为PIGN。由于脑病的存在,怀疑PRES继发于高血压急症,经脑部MRI证实。他还表现出自身免疫性溶血性贫血,血红蛋白高达5克/分升。治疗以降压治疗、神经保护措施和类固醇治疗为基础。患者住院31天后出院,出院后6个月无症状。结论:在PIGN的发展过程中,在出现非特异性神经系统症状之前,必须有较高的PRES怀疑指数。现有的贫血有时可能是自身免疫性的。
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[Posterior reversible encephalopathy syndrome (PRES) and hemolytic anemia: two severe and unusual complications of acute post-streptococcal glomerulonephritis].

The most frequent cause of nephritic syndrome in the pediatric population is acute post-infectious glomerulonephritis (PIGN). A rare complication is posterior reversible encephalopathy syndrome (PRES), characterized by subcortical vasogenic cerebral edema associated with variable neurological symptoms. The development of autoimmune hemolytic anemia is an atypical clinical presentation.

Objective: To report the coexistence of two unusual and serious extrarenal complications of PIGN and to discuss potential mechanisms involved in their development.

Clinical case: A 4-year-old male patient, with a 5-day history of hematuria and edema, headache, nausea, and vomiting. He was admitted in convulsive status and hypertensive crisis, laboratory showed C3 hypocomplementemia and high titers of Antistreptolysin O, which was interpreted as PIGN. Due to the presence of encephalopathy, PRES secondary to hypertensive emergency was suspected, which was confirmed by brain MRI. He also presented autoimmune hemolytic anemia, with hemoglobin up to 5 g/dL. The treatment was based on antihypertensive therapy, neuroprotective measures, and steroid treatment. He was discharged 31 days after hospitalization and remained asymptomatic 6 months after discharge.

Conclusions: There must be a high suspicion index of PRES before the appearance of nonspecific neurological symptoms during the evolution of a PIGN. The existing anemia on occasion can be autoimmune.

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