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皮下注射蛋白C浓缩物治疗纯合子蛋白C缺乏症。

Treatment of homozygous protein C deficiency with subcutaneous protein C concentrate.

作者信息

Minford A M, Parapia L A, Stainforth C, Lee D

机构信息

St Luke's Hospital, Bradford.

出版信息

Br J Haematol. 1996 Apr;93(1):215-6. doi: 10.1046/j.1365-2141.1996.4691021.x.

DOI:10.1046/j.1365-2141.1996.4691021.x
PMID:8611462
Abstract

Subcutaneous protein C concentrate (Immuno, Vienna) was used to treat a child with homozygous protein C deficiency who was formerly treated with intravenous protein C concentrate. After 3000 units subcutaneous protein C concentrate (250 iu/kg), protective protein C levels were maintained for 48 h after infusion, with peak levels at 12 h. Subcutaneous protein C concentrate is given every third day and is well tolerated by the patient. No thrombotic events have occurred. We conclude that subcutaneous administration of protein C concentrate is a valuable therapeutic option in the long-term management of homozygous protein C deficiency and avoids the potential hazards of long-term central venous lines.

摘要

皮下注射蛋白C浓缩物(Immuno,维也纳)用于治疗一名纯合子蛋白C缺乏症儿童,该儿童此前接受静脉注射蛋白C浓缩物治疗。皮下注射3000单位蛋白C浓缩物(250 iu/kg)后,输注后48小时内可维持保护性蛋白C水平,12小时达到峰值水平。皮下注射蛋白C浓缩物每三天给药一次,患者耐受性良好。未发生血栓事件。我们得出结论,皮下注射蛋白C浓缩物是纯合子蛋白C缺乏症长期管理中的一种有价值的治疗选择,可避免长期中心静脉置管的潜在风险。

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Case Report: Successful Long-Term Management of a Low-Birth Weight Preterm Infant With Compound Heterozygous Protein C Deficiency With Subcutaneous Protein C Concentrate Up to Adolescence.
病例报告:低出生体重早产复合杂合子蛋白C缺乏婴儿采用皮下注射蛋白C浓缩物进行长期成功管理直至青春期
Front Pediatr. 2021 Sep 28;9:591052. doi: 10.3389/fped.2021.591052. eCollection 2021.
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