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[前激肽释放酶(弗莱彻因子)缺乏与活化部分凝血活酶时间反应延长]

[Prekallikrein (Fletcher Factor) Deficiency and Prolongation of APTT Reaction].

作者信息

Dietzel Hermann, Lutze Gerd, Kätzel Ralph, Liebscher Karin

机构信息

Institut für Transfusionsmedizin und klinische Hämostaseologie, Städtisches Klinikum "St. Georg", Leipzig.

出版信息

Med Klin (Munich). 2003 Oct 15;98(10):587-90. doi: 10.1007/s00063-003-1299-0.

Abstract

BACKGROUND

Prekallikrein (Fletcher factor) is a protein of the contact phase of the blood coagulation system. A deficiency is a very rare event. The clinical meaning is still unknown.

CASE REPORT

In a 24-year-old patient of Croatian nationality, who has never suffered from any kind of proneness to hemorrhage, a considerable prolongation of activated partial thromboplastin time (APTT) was diagnosed preoperatively. The prothrombin time (PT) was normal. The patient had to be subjected to horseshoe-kidney surgery. CLOTTING TESTS: A very seldom Fletcher factor (prekallikrein) deficiency type II was detected. The activity was < 1%, but the prekallikrein concentration was normal, comparable to that in normal plasma.

THERAPY AND COURSE

Complications were neither noticed during renal surgery nor postoperatively. The patient did not show clinical symptoms. This fact corroborates reports about similar cases published earlier.

CONCLUSION

Nevertheless, attention has to be paid to these exceptionally rare symptoms, as in some publications, thromboembolic or even bleeding complications were reported.

摘要

背景

前激肽释放酶(弗莱彻因子)是血液凝固系统接触相的一种蛋白质。缺乏这种蛋白质是非常罕见的情况。其临床意义仍不明确。

病例报告

一名24岁的克罗地亚籍患者,既往从未有过任何出血倾向,术前诊断出活化部分凝血活酶时间(APTT)显著延长。凝血酶原时间(PT)正常。该患者必须接受马蹄肾手术。凝血试验:检测到非常罕见的II型弗莱彻因子(前激肽释放酶)缺乏。活性<1%,但前激肽释放酶浓度正常,与正常血浆中的浓度相当。

治疗与病程

肾手术期间及术后均未发现并发症。患者未出现临床症状。这一事实证实了此前发表的关于类似病例的报道。

结论

然而,必须关注这些极其罕见的症状,因为在一些出版物中,曾报道过血栓栓塞甚至出血并发症。

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