迟做总比不做好:复发性非典型溶血性尿毒症综合征的诊断。

IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY
Sari Aaltonen, Anne Räisänen-Sokolowski, Kati Kaartinen
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引用次数: 0

摘要

非典型溶血性尿毒症综合征(aHUS)是一种罕见的血栓性微血管病(TMA),包括微血管性溶血性贫血、消耗性血小板减少症和终末器官损伤。由于溶血性尿毒综合征通常表现在原生肾脏和移植肾脏,因此终末期肾脏疾病的风险增加。在移植中,虽然可以看到新生疾病,但复发性疾病更为常见。病因是可变的,有原发性的也有继发性的。aHUS往往构成诊断和治疗的挑战,这可能导致诊断和治疗的相当大的延误。在过去的几十年里,在了解这种毁灭性疾病的机制和治疗选择方面取得了巨大进展。我们报告一位50岁的女性,她在9岁时接受了母亲的第一次肾脏移植。她经历了反复的移植失败,直到第四次移植失败后,aHUS的诊断才变得明显。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Better late than never: Diagnosis of recurrent atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) comprising microangiopathic hemolytic anemia, consumptive thrombocytopenia, and end-organ damage. Risk of end-stage renal disease is increased as HUS usually manifests in native and transplanted kidneys. In transplants, while de novo disease can be seen, recurrent disease is more common. The etiology is variable, being either primary or secondary. aHUS often constitutes a diagnostic and therapeutic challenge, which may lead to a considerable delay in the diagnosis and treatment. During the last decades, great progress has been made in understanding the mechanisms and therapeutic options of this devastating condition. We present a case of a 50-year-old female who received her first kidney transplant from her mother at the age of 9 years. She experienced recurrent losses of transplants, and only after the loss of her fourth transplant did the diagnosis of aHUS become evident.

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来源期刊
Clinical nephrology
Clinical nephrology 医学-泌尿学与肾脏学
CiteScore
2.10
自引率
9.10%
发文量
138
审稿时长
4-8 weeks
期刊介绍: Clinical Nephrology appears monthly and publishes manuscripts containing original material with emphasis on the following topics: prophylaxis, pathophysiology, immunology, diagnosis, therapy, experimental approaches and dialysis and transplantation.
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