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幼儿期维生素K依赖因子缺乏的研究,特别涉及凝血酶原活性和抗原水平。

Studies on vitamin K-dependent factor deficiency during early childhood with special reference to prothrombin activity and antigen level.

作者信息

Fujimura Y, Mimura Y, Kinoshita S, Yoshioka A, Kitawaki T, Yoshioka K, Takamiya O

出版信息

Haemostasis. 1982;11(2):90-5. doi: 10.1159/000214646.

Abstract

8 young infants aged 14 days to 5 months with vitamin K-dependent factor deficiency were studied with special reference to prothrombin activity and antigen level. Among them, 3 infants had congenital bile duct atresia and 5 were breast-fed babies with severe hemorrhagic tendency of unknown cause. In the patients with both congenital bile duct atresia and breast feeding the ratio of prothrombin activity to prothrombin antigen was lower than 0.1. Furthermore, the arc of prothrombin antigen in these patients demonstrated a faster anodal shift than did normal prothrombin antigen on crossed immunoelectrophoresis. This abnormal prothrombin antigen was not consumed after recalcification of patient plasma, and absorbed poorly on BaSO4. In addition, the abnormal prothrombin antigen disappeared from the patient plasma within a few days after parenteral administration of vitamin K. These results suggest that this abnormal prothrombin is PIVKA-II.

摘要

对8例年龄在14天至5个月的维生素K依赖因子缺乏的婴儿进行了研究,特别关注凝血酶原活性和抗原水平。其中,3例婴儿患有先天性胆管闭锁,5例为母乳喂养的婴儿,有原因不明的严重出血倾向。在患有先天性胆管闭锁且为母乳喂养的患者中,凝血酶原活性与凝血酶原抗原的比值低于0.1。此外,在交叉免疫电泳中,这些患者的凝血酶原抗原弧比正常凝血酶原抗原表现出更快的阳极迁移。这种异常的凝血酶原抗原在患者血浆重新钙化后未被消耗,并且在硫酸钡上的吸附较差。另外,在肠胃外给予维生素K后的几天内,异常的凝血酶原抗原从患者血浆中消失。这些结果表明这种异常的凝血酶原是异常凝血酶原-II(PIVKA-II)。

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