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强直性肌营养不良中的下丘脑 - 垂体功能。

Hypothalamic-pituitary function in myotonic dystrophy.

作者信息

Mahler C, Parizel G

出版信息

J Neurol. 1982;226(4):233-42. doi: 10.1007/BF00313396.

Abstract

Function of the hypothalamic-pituitary axis was investigated in seven patients with myotonic dystrophy (MD). HGH and ACTH secretion were normal. TSH response to TRH was impaired in about half the cases, without concomitant thyroid dysfunction. LH and FSH levels were often elevated, with inconsistent response to LH-RH stimulation, Gonadotrophin disturbances in MD have previously been attributed to a primary gonadal lesion, characteristically seen in this disease. High prolactin levels in six of our seven patients however suggest that gonadal failure may be also be due to hyperprolactinemia through the direct anti-gonadal effect of prolactin and its interference with hypothalamic-pituitary regulation of gonadotrophin secretion.

摘要

对7例强直性肌营养不良(MD)患者的下丘脑 - 垂体轴功能进行了研究。生长激素(HGH)和促肾上腺皮质激素(ACTH)分泌正常。约半数病例促甲状腺激素(TSH)对促甲状腺激素释放激素(TRH)的反应受损,但无伴随的甲状腺功能障碍。促黄体生成素(LH)和促卵泡生成素(FSH)水平常升高,对促黄体生成素释放激素(LH - RH)刺激的反应不一致。MD中的促性腺激素紊乱以前被归因于原发性性腺病变,这在该疾病中很典型。然而,我们7例患者中有6例催乳素水平升高,提示性腺功能衰竭也可能是由于催乳素的直接抗性腺作用及其对下丘脑 - 垂体促性腺激素分泌调节的干扰导致的高催乳素血症。

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