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血色素沉着症中血清促性腺激素和睾酮对脉冲式促性腺激素释放激素无反应,提示存在垂体缺陷。

Non-responsiveness of serum gonadotropins and testosterone to pulsatile GnRH in hemochromatosis suggesting a pituitary defect.

作者信息

Duranteau L, Chanson P, Blumberg-Tick J, Thomas G, Brailly S, Lubetzki J, Schaison G, Bouchard P

机构信息

Service de Médecine Interne-Endocrinologie, Hôpital Lariboisière, Paris, France.

出版信息

Acta Endocrinol (Copenh). 1993 Apr;128(4):351-4. doi: 10.1530/acta.0.1280351.

DOI:10.1530/acta.0.1280351
PMID:8498154
Abstract

We investigated the potential pituitary origin of gonadal insufficiency in hemochromatosis. Gonadotropin secretion was studied in seven patients with hemochromatosis and hypogonadism, before and after chronic pulsatile GnRH therapy. Pulsatile LH secretion was studied before (sampling every 10 min for 6 h) and after 15-30 days of chronic pulsatile GnRH therapy (10-12 micrograms per pulse). Prior to GnRH therapy, all the patients had low serum testosterone, FSH and LH levels. LH secretion was non-pulsatile in four patients, while a single pulse was detected in the remaining three. Chronic pulsatile GnRH administration did not increase serum testosterone levels; similarly, serum LH levels remained low: neither pulse frequency nor pulse amplitude was modified. We conclude that hypogonadism in hemochromatosis is due to pituitary lesions.

摘要

我们研究了血色素沉着症中性腺功能不全的潜在垂体起源。在7例血色素沉着症伴性腺功能减退的患者中,于慢性脉冲式GnRH治疗前后对促性腺激素分泌进行了研究。在慢性脉冲式GnRH治疗(每脉冲10 - 12微克)前(每10分钟采样1次,共6小时)和治疗15 - 30天后,对LH脉冲分泌进行了研究。在GnRH治疗前,所有患者的血清睾酮、FSH和LH水平均较低。4例患者的LH分泌无脉冲,而其余3例仅检测到1个脉冲。慢性脉冲式GnRH给药未提高血清睾酮水平;同样,血清LH水平仍较低:脉冲频率和脉冲幅度均未改变。我们得出结论,血色素沉着症中的性腺功能减退是由垂体病变所致。

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引用本文的文献

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Endocrine dysfunction in hereditary hemochromatosis.遗传性血色素沉着症中的内分泌功能障碍。
J Endocrinol Invest. 2016 Aug;39(8):837-47. doi: 10.1007/s40618-016-0451-7. Epub 2016 Mar 7.
2
The Anterior Pituitary in Hemochromatosis.血色素沉着症中的垂体前叶。
Endocr Pathol. 1996 Summer;7(2):159-164. doi: 10.1007/BF02739976.
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Haemochromatosis as an endocrine cause of subfertility.血色素沉着症作为内分泌性不孕的一个病因
BMJ. 1998 Mar 21;316(7135):915-6. doi: 10.1136/bmj.316.7135.915.
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Transferrin and transferrin receptor in human hypophysis and pituitary adenomas.人垂体及垂体腺瘤中的转铁蛋白和转铁蛋白受体
Am J Pathol. 1998 Feb;152(2):413-22.