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[克兰费尔特综合征与腿部溃疡:凝血功能障碍的作用]

[Klinefelter's syndrome and leg ulcers: role of blood coagulation disorders].

作者信息

Hesse S, Berbis P, Juhan-Vague I, Bonerandi J J, Privat Y

机构信息

Clinique Dermatologique, Hôtel-Dieu, Marseille.

出版信息

Ann Dermatol Venereol. 1992;119(12):951-7.

PMID:1304686
Abstract

Genetically, Klinefelter's syndrome is characterized by a super-numerary chromosome X in male subjects presenting with clinical and biochemical hypoandrogenism and relative hyperoestrogenism. The association of Klinefelter's syndrome with chronic leg ulcer has given rise to numerous publications. The cause of the trophic disorders is unclear. We report two cases of this syndrome associated with chronic leg ulcers and anomalies of haemostasis. One of these two patients had platelet hyperaggregability followed by disorders of fibrinolysis, while the other had disorders of fibrinolysis. We reviewed the literature concerning the various aetiopathogenic hypotheses put forward to explain the cause of these leg ulcers, as well as their interactions and relationship with the hormonal anomalies. The position occupied by disorders of haemostasis is particularly developed.

摘要

从遗传学角度来看,克兰费尔特综合征的特征是男性患者存在一条额外的X染色体,伴有临床和生化方面的雄激素缺乏以及相对的雌激素过多。克兰费尔特综合征与慢性腿部溃疡的关联已引发了众多出版物。营养障碍的原因尚不清楚。我们报告了两例该综合征与慢性腿部溃疡及止血异常相关的病例。这两名患者中的一名存在血小板高聚集性,随后出现纤维蛋白溶解障碍,而另一名则有纤维蛋白溶解障碍。我们回顾了有关为解释这些腿部溃疡病因所提出的各种发病机制假说的文献,以及它们与激素异常的相互作用和关系。特别阐述了止血障碍所起的作用。

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