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食物依赖型周期性激素生成的库欣综合征

Cushing syndrome with food-dependent periodic hormonogenesis.

作者信息

Hamet P, Larochelle P, Franks D J, Cartier P, Bolte E

机构信息

Clinical Research Institute of Montreal, Quebec.

出版信息

Clin Invest Med. 1987 Nov;10(6):530-3.

PMID:2831001
Abstract

Periodic hormonogenesis has been described in patients with ACTH-dependent hypercorticism, and fluctuations of cortisol secretion have also been observed in patients with adrenal tumors. In this report, we studied a 41-year-old white male who presented with hypertension, central obesity, and muscle weakness of 2-years duration. His plasma cortisol was low (4.5 micrograms) in the morning and high in the evening (29.3 micrograms). Urinary free cortisol was 750 micrograms/day. A 24-hour cycle demonstrated highest values at noon and in late afternoon. This pattern was not suppressed by dexamethasone. When the patient was kept fasting, plasma cortisol remained low all day, and became elevated immediately after meal administration overnight. A left-sided adrenal mass was demonstrated and removed. In vitro, the adenylate cyclase activity of tumor tissue demonstrated more significant response to vasopressin than to ACTH; other tested peptides were inactive. We propose that a humoral factor induced by eating was responsible for the periodic hormonogenesis, directly stimulating the adrenal secretion of cortisol.

摘要

周期性激素生成现象已在促肾上腺皮质激素(ACTH)依赖性皮质醇增多症患者中有所描述,肾上腺肿瘤患者也观察到皮质醇分泌的波动。在本报告中,我们研究了一名41岁的白人男性,他患有高血压、中心性肥胖及持续2年的肌无力。其早晨血浆皮质醇水平较低(4.5微克),晚上较高(29.3微克)。尿游离皮质醇为750微克/天。24小时周期显示中午和傍晚时的值最高。这种模式未被地塞米松抑制。当患者禁食时,血浆皮质醇全天保持低水平,进食后过夜血浆皮质醇立即升高。发现并切除了左侧肾上腺肿块。体外实验中,肿瘤组织的腺苷酸环化酶活性对血管加压素的反应比对ACTH更显著;其他测试的肽无活性。我们提出,进食诱导的一种体液因子是周期性激素生成的原因,它直接刺激肾上腺分泌皮质醇。

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