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儿童阵发性睡眠性血红蛋白尿症致急性肾损伤 1 例

Pediatric Paroxysmal Nocturnal Hemoglobinuria Presenting as Acute Kidney Injury.

机构信息

Virginia Commonwealth University School of Medicine.

Departments of Pediatric Nephrology.

出版信息

J Pediatr Hematol Oncol. 2021 May 1;43(4):e543-e545. doi: 10.1097/MPH.0000000000001847.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterized by variable and diverse symptoms including the classic triad of hemolytic anemia, thrombosis, and bone marrow failure. It is a disorder primarily seen in the adult population. The authors report a unique case of an 8-year-old girl diagnosed with PNH after initially presenting with a febrile illness and acute kidney injury. Though rare in children, PNH should remain in the differential diagnosis of a child presenting with acute kidney injury. The disease has serious long-term complications, mandating timely diagnosis and appropriate therapy.

摘要

阵发性睡眠性血红蛋白尿症(PNH)是一种罕见的疾病,其特征是多变和多样的症状,包括溶血性贫血、血栓形成和骨髓衰竭的经典三联征。它主要发生在成年人群中。作者报告了一例 8 岁女孩的独特病例,该患者最初表现为发热性疾病和急性肾损伤,后被诊断为 PNH。尽管在儿童中罕见,但 PNH 仍应作为儿童急性肾损伤的鉴别诊断。该疾病有严重的长期并发症,需要及时诊断和适当的治疗。

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