Piperno A, Rivolta M R, D'Alba R, Fargion S, Rovelli F, Ghezzi A, Micheli M, Fiorelli G
Istituto di Scienze Biomediche S. Gerardo, Università di Milano, Monza, Italy.
J Endocrinol Invest. 1992 Jun;15(6):423-8. doi: 10.1007/BF03348765.
We studied endocrine functions at baseline and after TRH and LHRH stimulation in a group of 7 young male patients with genetic hemochromatosis (HE) without liver damage (i.e. fibrosis and cirrhosis). In five patients endocrine re-evaluations after complete iron depletion was also performed. Mean basal testosterone (T), FSH, LH and PRL were significantly lower than in controls. Serum T increased normally after HCG stimulation. The normal or high increments of LH after LHRH stimulation suggest that secretion capacity of LH was intact and that hypothalamic dysfunction could be responsible for the preclinical gonadal deficiency found in our patients. The response of PRL to TRH indicates that secretion capacity of lactotrophs although present, was decreased and did not improve after phlebotomy therapy. After iron depletion the two patients with the lowest basal T levels showed the highest increments indicating that in the early stages of hypothalamic-pituitary damage gonadal dysfunction is still reversible in HE patients.
我们对一组7名无肝损伤（即无纤维化和肝硬化）的年轻男性遗传性血色素沉着症（HE）患者在基线时以及促甲状腺激素释放激素（TRH）和促黄体生成素释放激素（LHRH）刺激后进行了内分泌功能研究。对其中5名患者在完全铁耗竭后也进行了内分泌重新评估。平均基础睾酮（T）、促卵泡生成素（FSH）、促黄体生成素（LH）和催乳素（PRL）显著低于对照组。人绒毛膜促性腺激素（HCG）刺激后血清T正常升高。LHRH刺激后LH正常或升高，提示LH的分泌能力完好，下丘脑功能障碍可能是我们患者中发现的临床前性腺功能减退的原因。PRL对TRH的反应表明，催乳细胞的分泌能力虽然存在，但有所下降，且放血治疗后未改善。铁耗竭后，基础T水平最低的两名患者升高幅度最大，表明在垂体下丘脑损伤的早期阶段，HE患者的性腺功能障碍仍然是可逆的。
