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一名青少年型血色素沉着症患者促性腺激素缺乏性性腺功能减退的可逆性

Reversibility of hypogonadotropic hypogonadism in a patient with the juvenile form of hemochromatosis.

作者信息

Angelopoulos Nicholas G, Goula Anastasia, Dimitriou Evagelos, Tolis George

机构信息

Endocrine Department, Hippokrateion Hospital of Athens, Athens, Greece.

出版信息

Fertil Steril. 2005 Dec;84(6):1744. doi: 10.1016/j.fertnstert.2005.05.070.

Abstract

OBJECTIVE

To report a case of complete reversibility of hypogonadotropic hypogonadism with intensive venesection treatment in juvenile hemochromatosis.

DESIGN

Case report.

SETTING

Endocrine department of Hippocrateion Hospital of Athens.

PATIENT(S): A 25-year-old man who presented with hypogonadotropic hypogonadism and severe iron overload due to juvenile hemochromatosis and who was initially treated with phlebotomies and androgen substitution.

INTERVENTION(S): Intensification of chelation therapy.

MAIN OUTCOME MEASURE(S): Clinical evaluation, serum ferritin concentration, and biochemical assessment of pituitary function were performed periodically.

RESULT(S): One year after normalization of serum ferritin levels and transferrin saturation was achieved, he became eugonadal.

CONCLUSION(S): We believe that hypogonadotropic hypogonadism in juvenile hemochromatosis may be reversible by a consequent venesection therapy probably because treatment was intensive and promptly introduced at a young age.

摘要

目的

报告一例青少年血色素沉着症患者,通过强化静脉放血治疗,其低促性腺激素性性腺功能减退完全可逆。

设计

病例报告。

地点

雅典希波克拉底翁医院内分泌科。

患者

一名25岁男性,因青少年血色素沉着症出现低促性腺激素性性腺功能减退和严重铁过载,最初接受静脉放血和雄激素替代治疗。

干预措施

强化螯合疗法。

主要观察指标

定期进行临床评估、血清铁蛋白浓度测定及垂体功能生化评估。

结果

血清铁蛋白水平和转铁蛋白饱和度恢复正常一年后,他性腺功能恢复正常。

结论

我们认为,青少年血色素沉着症所致的低促性腺激素性性腺功能减退可能通过后续的静脉放血疗法逆转,这可能是因为治疗强度大且在年轻时及时开始。

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