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An endocrine study of six patients with cerebral gigantism (Sotos' syndrome)

机译:对六名脑巨人症(Sotos综合征)患者的内分泌研究

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摘要

The auxological and clinical basis for the diagnosis will be shown. Basal prolactin levels were measured and a prolonged glucose tolerance test, an L-dopa test, a TRH test and an LH-RH test performed. Abnormalities were found in five of the six patients. These included high basal prolactin levels, a pre-diabetic blood sugar curve, inadequate suppression of growth hormone or a paradoxical rise in growth hormone in response to glucose, an excessive or continuing rise of TSH following TRH, an excessive response of prolactin to TRH, and, in one child, a pubertal response of LH and FSH to LH-RH. The endocrine abnormalities demonstrated, although not consistent, suggest possible hypothalamic dysfunction in cerebral gigantism, in which a variable aetiology is likely.
机译:将显示诊断的皮肤科和临床基础。测量基础催乳素水平,并进行延长的葡萄糖耐量试验,L-多巴试验,TRH试验和LH-RH试验。在六名患者中的五名中发现异常。其中包括高的基础催乳素水平,糖尿病前血糖曲线,对葡萄糖的生长激素抑制不足或生长激素反常升高,TRH后TSH过度或持续升高,催乳素对TRH的过度反应,在一个孩子中,LH和FSH对LH-RH的青春期反应。已证明的内分泌异常虽然不一致,但提示在脑巨人症中可能存在下丘脑功能障碍,其中病因可能会发生变化。

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