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成年庞贝病患者脱敏治疗史及对阿糖苷酶α的过敏反应。

Desensitization of an adult patient with Pompe disease and a history of anaphylaxis to alglucosidase alfa.

机构信息

Division of Medical Genetics, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA 22908-0386, USA.

出版信息

Mol Genet Metab. 2009 Nov;98(3):319-21. doi: 10.1016/j.ymgme.2009.07.001. Epub 2009 Jul 7.

DOI:10.1016/j.ymgme.2009.07.001
PMID:19640753
Abstract

We report on the successful desensitization of an adult female with Pompe disease who had previously experienced anaphylaxis to intravenous alglucosidase alfa therapy. The starting alglucosidase alfa dose for desensitization was 10mg/kg with gradual dose escalation and desensitization via serial dilution was completed over five infusions. This methodology serves as a means to desensitize patients with prior anaphylactic response to alglucosidase alfa so that enzyme replacement therapy can be utilized.

摘要

我们报告了一例成功脱敏的成年女性 Pompe 病患者,她之前曾对静脉用阿糖苷酶α治疗发生过过敏反应。脱敏起始阿糖苷酶α剂量为 10mg/kg,逐渐递增剂量,并通过连续稀释分五次输注完成脱敏。这种方法可用于对阿糖苷酶α有既往过敏反应的患者进行脱敏,从而可以使用酶替代疗法。

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