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一名儿童频繁复发的溶血尿毒综合征的血浆置换治疗

Plasma exchanges in frequently recurrent hemolytic-uremic syndrome in a child.

作者信息

Feldhoff C M, Luboldt W, Bussmann K, Schrör K

出版信息

Int J Pediatr Nephrol. 1983 Dec;4(4):239-42.

PMID:6363316
Abstract

22 plasma exchanges (PE) were carried out on a 9 year old boy with frequently recurrent hemolytic-uremic syndrome (HUS) and persistently low plasma factor which normally stimulates vascular prostacyclin (PGI2) release. The factor was partially restored after PE but declined within 2-4 weeks. No major recurrence of HUS occurred, but "minor" attacks recurred despite PE. It is likely that a genetic predisposition is responsible for the recurrences of HUS, but the ultimate relationship between recurrent HUS and persistent plasma deficiency for PGI2-release remains to be defined.

摘要

对一名9岁男孩进行了22次血浆置换(PE),该男孩患有频繁复发的溶血尿毒综合征(HUS),且血浆中通常刺激血管前列环素(PGI2)释放的因子持续处于低水平。血浆置换后该因子部分恢复,但在2 - 4周内又下降。溶血尿毒综合征未出现重大复发,但尽管进行了血浆置换,“轻微”发作仍有复发。溶血尿毒综合征的复发可能是由遗传易感性导致的,但复发性溶血尿毒综合征与PGI2释放的持续性血浆缺乏之间的最终关系仍有待确定。

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