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特纳综合征中的非典型生化检查结果:一个可能亚组的识别

Atypical biochemical findings in Turner's syndrome: identification of a possible subset.

作者信息

Valenta L J, Elias A N, Bocian M

出版信息

Fertil Steril. 1984 Nov;42(5):798-802. doi: 10.1016/s0015-0282(16)48211-7.

DOI:10.1016/s0015-0282(16)48211-7
PMID:6436075
Abstract

Two patients with Turner's syndrome, Hashimoto's thyroiditis, and partial intrasellar arachnoidocele (empty sella) are described. Both patients had low basal and LH-RH-stimulated gonadotropin release and moderately exaggerated TSH and PRL response to insulin-induced hypoglycemia and TRH. Such findings are typical of a disturbance of the hypothalamic-pituitary axis. In our patients, these findings could be due to the presence of the intrasellar arachnoidocele or might represent a loss of the central dopaminergic tone possibly as the consequence of an autoimmune process. It remains to be established whether the frequency of intrasellar arachnoidocele and Hashimoto's thyroiditis in patients with Turner's syndrome justifies classification of the condition of these patients as a distinct subtype.

摘要

本文描述了两名患有特纳综合征、桥本甲状腺炎和部分鞍内蛛网膜囊肿(空蝶鞍)的患者。两名患者基础状态下以及促性腺激素释放激素(LH-RH)刺激后的促性腺激素释放均较低,对胰岛素诱导的低血糖和促甲状腺激素释放激素(TRH)的促甲状腺激素(TSH)和催乳素(PRL)反应适度增强。这些发现是下丘脑-垂体轴功能紊乱的典型表现。在我们的患者中,这些发现可能是由于鞍内蛛网膜囊肿的存在,也可能代表中枢多巴胺能张力的丧失,这可能是自身免疫过程的结果。特纳综合征患者中鞍内蛛网膜囊肿和桥本甲状腺炎的发生率是否足以将这些患者的病情归类为一种独特的亚型,仍有待确定。

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1
Atypical biochemical findings in Turner's syndrome: identification of a possible subset.特纳综合征中的非典型生化检查结果:一个可能亚组的识别
Fertil Steril. 1984 Nov;42(5):798-802. doi: 10.1016/s0015-0282(16)48211-7.
2
The effect of contraceptive steroids on hypothalamic-pituitary function.避孕类固醇对下丘脑 - 垂体功能的影响。
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Turner's syndrome associated with Hashimoto's thyroiditis and sarcoidosis: a case report.特纳综合征合并桥本甲状腺炎和结节病:一例报告
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引用本文的文献

1
Turner syndrome and postpubertal Empty sella syndrome: a case report and literature review.特纳综合征与青春期后空蝶鞍综合征:一例报告及文献综述
Front Endocrinol (Lausanne). 2025 Jun 3;16:1552724. doi: 10.3389/fendo.2025.1552724. eCollection 2025.
2
X-ring Turner's syndrome with combined immunodeficiency and selective gonadotropin defect.
J Endocrinol Invest. 1989 Apr;12(4):257-63. doi: 10.1007/BF03349979.