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儿童肾病综合征伴脑桥外髓鞘溶解症。

Extrapontine myelinolysis in a child with nephrotic syndrome.

机构信息

Department of Pediatrics, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Maharashtra, Mumbai, India.

出版信息

Pediatr Neurol. 2010 Aug;43(2):139-41. doi: 10.1016/j.pediatrneurol.2010.04.006.

Abstract

Extrapontine myelinolysis is rare in children. We describe a 6-year-old girl with nephrotic syndrome who presented with symptomatic hyponatremia, and who developed acute quadriparesis with pseudobulbar palsy during rapid correction of the hyponatremia. Cranial magnetic resonance imaging demonstrated bilateral, symmetric basal ganglia lesions (extrapontine myelinolysis). The extrapontine myelinolysis was caused by rapid correction of severe and prolonged hyponatremia with intravenous 3% sodium chloride. The child demonstrated complete neurologic recovery. Prevention of this rare condition involves recognizing patients at risk for the disorder, and avoiding rapid correction of severe and prolonged hyponatremia. To the best of our knowledge, this is the first case report of extrapontine myelinolysis in a child with nephrotic syndrome.

摘要

桥外髓鞘溶解症在儿童中较为罕见。我们描述了一例肾病综合征患儿,其因低钠血症出现症状性低钠血症,并在快速纠正低钠血症时发生急性四肢瘫痪伴假性延髓性麻痹。头颅磁共振成像显示双侧、对称基底节病变(桥外髓鞘溶解症)。桥外髓鞘溶解症是由静脉输注 3%氯化钠快速纠正严重和长期的低钠血症引起的。患儿完全恢复了神经功能。预防这种罕见情况需要识别有这种疾病风险的患者,并避免快速纠正严重和长期的低钠血症。据我们所知,这是首例肾病综合征患儿桥外髓鞘溶解症的病例报告。

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