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蛋白C浓缩物在新生儿期的应用。

Use of protein C concentrate in neonatal period.

作者信息

De Carolis M P

机构信息

Divisione di Neonatologia, Università Cattolica del S. Cuore, Roma.

出版信息

Minerva Pediatr. 2010 Jun;62(3 Suppl 1):29-30.

Abstract

Levels of protein C, low at birth, physiologically Increase until six months of age and achieve the adult range after puberty. Protein C deficiency may be congenital or acquired. Severe protein C deficiency is a rare autosomal recessive disorder that usually presents in neonatal period with purpura fulminans. Acquired protein C deficiency may be caused by increased consumption (e.g., asphyxia, overt DIC, severe infection without overt DIC, acute VTE) or by decreased synthesis of the active carboxylated protein (e.g. administration of vitamin K antagonists, severe hepatic synthetic disfunction). Two different formulations of protein C are available: recombinant human activated protein C (rhAPC) and human plasma-derived viral-inactivated protein C. It is known that in septic patients replacement therapy with rhAPC reduces mortality but is associated with an increased risk of bleeding. During the neonatal period, when a higher risk of bleeding exists, the human plasma-derived viral-inactivated protein C concentrate may represent an effective therapeutic option. In fact, its administration results effective both in severe congenital and acquired forms of protein C deficiency.

摘要

蛋白C水平在出生时较低,在生理上会一直增加直至6个月大,并在青春期后达到成人水平。蛋白C缺乏症可能是先天性的或后天获得性的。严重的蛋白C缺乏症是一种罕见的常染色体隐性疾病,通常在新生儿期表现为暴发性紫癜。后天获得性蛋白C缺乏症可能是由于消耗增加(如窒息、明显的弥散性血管内凝血、无明显弥散性血管内凝血的严重感染、急性静脉血栓栓塞)或活性羧化蛋白合成减少(如使用维生素K拮抗剂、严重的肝脏合成功能障碍)引起的。有两种不同的蛋白C制剂:重组人活化蛋白C(rhAPC)和人血浆来源的病毒灭活蛋白C。已知在脓毒症患者中,用rhAPC进行替代治疗可降低死亡率,但会增加出血风险。在新生儿期,当出血风险较高时,人血浆来源的病毒灭活蛋白C浓缩物可能是一种有效的治疗选择。事实上,它的给药对严重的先天性和后天获得性蛋白C缺乏症均有效。

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