Siminoski K, D'Costa M, Walfish P G
Department of Medicine, Mount Sinai Hospital and Medical School, University of Toronto, Ontario, Canada.
J Endocrinol Invest. 1990 Nov;13(10):849-53. doi: 10.1007/BF03349640.
Hypogonadism is a common finding in idiopathic hemochromatosis. Most studies have localized the defect to either the pituitary gland or the testes. We describe a case with evidence that favors the likely concomitant involvement of the hypothalamus as a factor in the observed hypogonadism. A clinically hypogonadal male with hemochromatosis had a low testosterone concentration with inappropriately normal serum LH levels. Leydig cell function was intact, as demonstrated by a normal increase in serum testosterone following HCG administration. However, although the pituitary secretion of LH was normal in response to GnRH stimulation, clomiphene administration did not produce an increase in LH and FSH, suggesting that there was a defect in the hypothalamic GnRH response. Since the FSH and prolactin responses to stimulatory testing were inadequate, coexisting pituitary dysfunction was likely also present. We conclude that this man had hypogonadism with laboratory evidence for a combined defect in hypothalamic and pituitary function.
性腺功能减退是特发性血色素沉着症的常见表现。大多数研究将缺陷定位在垂体或睾丸。我们描述了一个病例，有证据表明下丘脑可能也参与其中，是观察到的性腺功能减退的一个因素。一名患有血色素沉着症且临床诊断为性腺功能减退的男性，睾酮浓度低，而血清促黄体生成素（LH）水平却正常得不合常理。人绒毛膜促性腺激素（HCG）给药后血清睾酮正常升高，表明睾丸间质细胞功能完好。然而，尽管GnRH刺激后垂体LH分泌正常，但克罗米芬给药并未使LH和促卵泡生成素（FSH）增加，提示下丘脑GnRH反应存在缺陷。由于FSH和催乳素对刺激试验的反应不足，可能同时存在垂体功能障碍。我们得出结论，该男性患有性腺功能减退，实验室证据表明下丘脑和垂体功能存在联合缺陷。
