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1型神经纤维瘤病中嗜铬细胞瘤的可乐定抑制试验

Clonidine suppression testing for pheochromocytoma in neurofibromatosis type 1.

作者信息

Wan WingYee, Nguyen Bichle, Graybill Sky, Kim Jonathan

机构信息

Internal Medicine, Brooke Army Medical Center, Fort Sam Houston, Texas, USA.

Endocrinology Department, Brooke Army Medical Center, Fort Sam Houston, Texas, USA.

出版信息

BMJ Case Rep. 2019 Jun 25;12(6):e228263. doi: 10.1136/bcr-2018-228263.

Abstract

Compared with the general population, rates of pheochromocytoma are higher in neurofibromatosis type 1 (NF1) patients. However, pheochromocytoma testing is often plagued by false positive results. Here we present a patient with NF1, elevated urinary metanephrine levels, and an indeterminate adrenal nodule. Clonidine suppression testing aided diagnosis and led to definitive surgical treatment that confirmed a pheochromocytoma. Pheochromocytoma screening and clonidine suppression testing can both aid in the evaluation for catecholamine-secreting tumours.

摘要

与普通人群相比,1型神经纤维瘤病(NF1)患者的嗜铬细胞瘤发病率更高。然而,嗜铬细胞瘤检测常常受到假阳性结果的困扰。在此,我们报告1例患有NF1、尿间甲肾上腺素水平升高且肾上腺结节性质不明的患者。可乐定抑制试验有助于诊断,并最终促成了确定性手术治疗,确诊为嗜铬细胞瘤。嗜铬细胞瘤筛查和可乐定抑制试验均有助于评估儿茶酚胺分泌性肿瘤。

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