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去氨加压素对甲型血友病携带者的输注:血浆凝血因子 VIII 和血管性血友病因子的不同表现

DDAVP infusion in haemophilia A carriers: different behaviour of plasma factor VIII and von Willebrand factor.

作者信息

Casonato A, Dannhauser D, Pontara E, Bertomoro A, Orazi B, Santarossa L, Zerbinati P, Girolami A

机构信息

University of Padma, Medical School, Institute of Medical Semeiotics, Italy.

出版信息

Blood Coagul Fibrinolysis. 1996 Jul;7(5):549-53.

PMID:8874865
Abstract

1-desamino-8-D-arginine vasopressin (DDAVP) increases factor VIII (FVIII) and von Willebrand factor (vWF) levels in patients with haemophilia A and in some patients with von Willebrand disease. It is generally held that the increase of FVIII is a consequence of the increase of vWF. Carriers of haemophilia A generally, but not always, show plasma FVIII levels lower than vWF due to an abnormality in one of the two alleles of the FVIII gene. We investigated the time-course of plasma FVIII:C and vWF:Ag levels in 25 obligate carriers of haemophilia A after DDAVP infusion. In carriers with a normal FVIII to vWF ratio (> 0.8), DDAVP induced a progressive ratio decrease that reached levels significantly lower than that taken as cut-off to discriminate between low and normal values (0.68 +/- 0.1 vs before 0.912 +/- 0.18). In carriers with a borderline (0.7-0.8) or reduced (< 0.7) ratio DDAVP induced a further decrease in the FVIII/vWF ratio, albeit with a different kinetic; after an initial increase, values were lower than pre-DDAVP figures. In all subjects, following the post-DDAVP peak, plasma FVIII progressively decreased while vWF contemporaneously continued to increase. In contrast, DDAVP did not induce significant changes in the FVIII/vWF ratio in normal females, and the two molecules appeared to increase similarly throughout the observation period. These findings indicate that after DDAVP, FVIII increases less or for a shorter time than vWF, also in haemophilia A carriers who have a normal FVIII/vWF ratio. Hence, DDAVP may help identify haemophilia A carriers, especially subjects with normal or borderline ratios. Even though molecular biology procedures at present are the best and more reliable tools to identify the carrier state, DDAVP seems to improve the accuracy of haemostatic parameters.

摘要

1-去氨基-8-D-精氨酸加压素(DDAVP)可提高甲型血友病患者以及部分血管性血友病患者的凝血因子VIII(FVIII)和血管性血友病因子(vWF)水平。一般认为,FVIII水平的升高是vWF水平升高的结果。甲型血友病携带者通常(但并非总是)由于FVIII基因两个等位基因之一的异常,其血浆FVIII水平低于vWF。我们研究了25名甲型血友病确诊携带者在输注DDAVP后血浆FVIII:C和vWF:Ag水平的时间变化过程。在FVIII与vWF比值正常(>0.8)的携带者中,DDAVP导致比值逐渐降低,降至显著低于用于区分低值和正常值得临界值(0.68±0.1,而给药前为0.912±0.18)。在比值处于临界范围(0.7 - 0.8)或降低(<0.7)的携带者中,DDAVP导致FVIII/vWF比值进一步降低,尽管动力学有所不同;在最初升高后,数值低于给药前水平。在所有受试者中,DDAVP给药后达到峰值后,血浆FVIII逐渐下降,而vWF同时继续升高。相比之下,DDAVP未在正常女性中引起FVIII/vWF比值的显著变化,并且在整个观察期内这两种分子的升高情况相似。这些发现表明,在使用DDAVP后,即使在FVIII/vWF比值正常的甲型血友病携带者中,FVIII的升高幅度也较小或持续时间较短。因此,DDAVP可能有助于识别甲型血友病携带者,尤其是比值正常或处于临界范围的受试者。尽管目前分子生物学方法是识别携带者状态的最佳且更可靠的工具,但DDAVP似乎提高了止血参数的准确性。

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