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多巴胺激动剂反应性库欣病

Dopamine agonist-responsive Cushing's disease.

作者信息

Anand Gurpreet, Bink Andrea, Beuschlein Felix, Schmid Christoph

机构信息

Department of Endocrinology, University Hospital Zürich, Zürich, Switzerland.

Department of Radiology, University Hospital Zürich, Zürich, Switzerland.

出版信息

BMJ Case Rep. 2019 Feb 13;12(2):e228045. doi: 10.1136/bcr-2018-228045.

Abstract

A 47-year-old Caucasian man was referred to our clinic with a severe clinical and biochemical phenotype of endogenous hypercortisolism for further evaluation and treatment. In addition to confirming adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, we found left temporal hemianopsia, massively increased prolactin, increased growth hormone/insulin-like growth factor 1 values, hypogonadotropic hypogonadism and central hypothyroidism. As the cause of these abnormalities we revealed an invasive macroadenoma of the pituitary secreting ACTH, prolactin and growth hormone, resulting not only in a clinically predominant picture of Cushing's syndrome but also causing hypogonadotropic hypogonadism and central hypothyroidism. The patient responded surprisingly well to dopamine agonist treatment leading not only to normalisation of prolactin levels but also to clinical and biochemical remission of Cushing's syndrome. Tumour size decreased successively in follow-up MRI scans. Despite lacking immunohistochemical analysis of tumour tissue, we assume plurihormonal secretion of ACTH, prolactin and growth hormone from pituitary macroadenoma, which fortunately responded well to dopamine agonist treatment.

摘要

一名47岁的白种男性因内源性皮质醇增多症的严重临床和生化表型被转诊至我们的诊所,以进行进一步评估和治疗。除了确诊促肾上腺皮质激素(ACTH)依赖性库欣综合征外,我们还发现患者存在左侧颞侧偏盲、催乳素大幅升高、生长激素/胰岛素样生长因子1值升高、低促性腺激素性性腺功能减退和中枢性甲状腺功能减退。作为这些异常情况的病因,我们发现了垂体的侵袭性大腺瘤,其分泌ACTH、催乳素和生长激素,不仅导致了临床上以库欣综合征为主的表现,还引起了低促性腺激素性性腺功能减退和中枢性甲状腺功能减退。该患者对多巴胺激动剂治疗反应出奇地好,不仅使催乳素水平恢复正常,还使库欣综合征在临床和生化方面得到缓解。在后续的磁共振成像(MRI)扫描中,肿瘤大小逐渐减小。尽管缺乏对肿瘤组织的免疫组化分析,但我们推测垂体大腺瘤分泌ACTH、催乳素和生长激素,幸运的是,该肿瘤对多巴胺激动剂治疗反应良好。

相似文献

1
Dopamine agonist-responsive Cushing's disease.多巴胺激动剂反应性库欣病
BMJ Case Rep. 2019 Feb 13;12(2):e228045. doi: 10.1136/bcr-2018-228045.
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PPAR-gamma in Cushing's disease.库欣病中的过氧化物酶体增殖物激活受体γ
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本文引用的文献

7
A rare case of ACTH-LH plurihormonal pituitary adenoma: letter to the editor.
Acta Neurochir (Wien). 2014 Jul;156(7):1389-91. doi: 10.1007/s00701-014-2027-7. Epub 2014 Feb 19.

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