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与遗传性血色素沉着症相关的低促性腺激素性性腺功能减退的可逆性。

Reversibility of hypogonadotrophic hypogonadism associated with genetic haemochromatosis.

作者信息

Cundy T, Butler J, Bomford A, Williams R

机构信息

Department of Medicine, Kings College School of Medicine and Dentistry, Denmark Hill, London, UK.

出版信息

Clin Endocrinol (Oxf). 1993 Jun;38(6):617-20. doi: 10.1111/j.1365-2265.1993.tb02143.x.

DOI:10.1111/j.1365-2265.1993.tb02143.x
PMID:8334747
Abstract

OBJECTIVE

Recent reports have suggested that hypogonadotrophic hypogonadism in men with genetic haemochromatosis can be reversed by aggressive venesection therapy. We have studied prospectively men with this complication of haemochromatosis in order to document the frequency and completeness of recovery from hypogonadism.

PATIENTS AND DESIGN

Six men with symptomatic hypogonadotrophic hypogonadism and genetic haemochromatosis were studied before beginning venesection therapy and again after depletion of body iron stores.

MEASUREMENT

Symptoms of hypogonadism, serum gonadotrophins and serum total and free testosterone concentrations.

RESULTS

Five men aged 47-66 showed no symptomatic improvement and no change in serum gonadotrophin or testosterone concentrations. The symptoms of one man, aged 33, resolved completely after venesection and this was accompanied by increases in serum LH and FSH. Serum total and free testosterone concentrations increased fourfold, but remained subnormal.

CONCLUSION

Only one partial recovery from hypogonadotrophic hypogonadism was seen and this was in the youngest subject. In reviewing the other documented cases of reversal it would appear that the age at diagnosis is critical and there are no proven cases of reversal of hypogonadotrophic hypogonadism in men over the age of 40 at the start of venesection therapy.

摘要

目的

最近的报告表明,遗传性血色素沉着症男性患者的低促性腺激素性性腺功能减退可通过积极的放血疗法得到逆转。我们对患有血色素沉着症这种并发症的男性进行了前瞻性研究,以记录性腺功能减退恢复的频率和完全程度。

患者与设计

对6名有症状的低促性腺激素性性腺功能减退且患有遗传性血色素沉着症的男性在开始放血治疗前以及身体铁储存耗竭后再次进行了研究。

测量

性腺功能减退的症状、血清促性腺激素以及血清总睾酮和游离睾酮浓度。

结果

5名年龄在47 - 66岁的男性症状未改善,血清促性腺激素或睾酮浓度也无变化。1名33岁男性的症状在放血后完全缓解,同时血清促黄体生成素(LH)和促卵泡生成素(FSH)升高。血清总睾酮和游离睾酮浓度增加了四倍,但仍低于正常水平。

结论

仅在最年轻的受试者中观察到低促性腺激素性性腺功能减退有部分恢复。回顾其他已记录的逆转病例,似乎诊断时的年龄至关重要,在开始放血治疗时年龄超过40岁的男性中,尚无低促性腺激素性性腺功能减退逆转的经证实病例。

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Reversibility of hypogonadotrophic hypogonadism associated with genetic haemochromatosis.与遗传性血色素沉着症相关的低促性腺激素性性腺功能减退的可逆性。
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