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典型血友病携带者中抗血友病因子(凝血因子VIII)促凝滴度和沉淀抗原水平的变异性来源。

Sources of variability in antihemophilic factor (factor VIII) procoagulant titers and precipitating antigen levels among obligate carriers of classic hemophilia.

作者信息

Jones P K, Ratnoff O D

出版信息

Blood. 1981 May;57(5):928-32.

PMID:6783148
Abstract

Chediak et al. have reported that the titer of procoagulant antihemophilic factor (AHF:C; factor VIII:C) was significantly lower in obligate carriers of classic hemophilia who were daughters of affected men (paternal carriers) than in those whose fathers were normal by history (maternal carriers). In contrast, among 113 obligate carriers of hemophilia, no significant difference in procoagulant AHF titers was observed between paternal and maternal carriers. The concentration of AHF-like precipitating antigens, however, was significantly higher in maternal than in paternal carriers. This difference may have reflected in part the greater severity of disease in affected males in the families of maternal carriers.

摘要

切迪亚克等人报告称,患血友病男性的女儿(父系携带者)这一典型血友病的必然携带者,其促凝血抗血友病因子(AHF:C;凝血因子VIII:C)滴度显著低于其父亲无血友病病史的携带者(母系携带者)。相比之下,在113名血友病必然携带者中,父系携带者和母系携带者的促凝血AHF滴度未观察到显著差异。然而,母系携带者中AHF样沉淀抗原的浓度显著高于父系携带者。这种差异可能部分反映了母系携带者家族中患病男性的病情更严重。

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