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肾上腺髓质疾病。

Diseases of the adrenal medulla.

作者信息

Fung M M, Viveros O H, O'Connor D T

机构信息

Department of Medicine and Center for Human Genetics and Genomics, University of California at San Diego, La Jolla, CA 92093-0838, USA.

出版信息

Acta Physiol (Oxf). 2008 Feb;192(2):325-35. doi: 10.1111/j.1748-1716.2007.01809.x. Epub 2007 Nov 16.

Abstract

The adrenal glands are vital in the organism's response to environmental stress. The outer cortex releases steroid hormones: glucocorticoids, mineralocorticoids and sex hormones, which are crucial to metabolism, inflammatory reactions and fluid homeostasis. The medulla is different developmentally, functionally and structurally. It co-releases catecholamines (primarily adrenaline and to some extent noradrenaline) as well as peptides by the all-or-none process of exocytosis from chromaffin granules, to aid in blood pressure and blood flow regulation, with regulated increments during the activation of the sympathetic nervous system. The co-released peptides function to regulate catecholamine release, blood vessel contraction and innate immune responses. Pathology within the adrenal medulla and the autonomic nervous system is primarily because of neoplasms. The most common tumour, called phaeochromocytoma when located in the adrenal medulla, originates from chromaffin cells and excretes catecholamines, but may be referred to as secreting paragangliomas when found in extra-adrenal chromaffin cells. Neoplasms, such as neuroblastomas and ganglioneuromas, may also be of neuronal lineage. We will also briefly discuss the catecholamine deficiency state.

摘要

肾上腺在机体对环境应激的反应中至关重要。肾上腺外层皮质释放类固醇激素:糖皮质激素、盐皮质激素和性激素,这些激素对新陈代谢、炎症反应和液体平衡至关重要。肾上腺髓质在发育、功能和结构上有所不同。它通过嗜铬颗粒的全或无式胞吐过程共同释放儿茶酚胺(主要是肾上腺素,在一定程度上还有去甲肾上腺素)以及肽类,以帮助调节血压和血流,在交感神经系统激活期间有调节性增加。共同释放的肽类起到调节儿茶酚胺释放、血管收缩和先天免疫反应的作用。肾上腺髓质和自主神经系统内的病理学主要是由肿瘤引起的。最常见的肿瘤,位于肾上腺髓质时称为嗜铬细胞瘤,起源于嗜铬细胞并分泌儿茶酚胺,但在肾上腺外嗜铬细胞中发现时可能被称为分泌性副神经节瘤。神经母细胞瘤和神经节瘤等肿瘤也可能起源于神经细胞系。我们还将简要讨论儿茶酚胺缺乏状态。

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J Clin Endocrinol Metab. 2007 Apr;92(4):1217-25. doi: 10.1210/jc.2006-1544. Epub 2007 Feb 6.
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Phaeochromocytoma, new genes and screening strategies.嗜铬细胞瘤、新基因与筛查策略
Clin Endocrinol (Oxf). 2006 Dec;65(6):699-705. doi: 10.1111/j.1365-2265.2006.02714.x.
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Familial nonsyndromic pheochromocytoma.家族性非综合征性嗜铬细胞瘤。
Ann N Y Acad Sci. 2006 Aug;1073:149-55. doi: 10.1196/annals.1353.015.
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Pheochromocytoma: presentation, diagnosis and treatment.嗜铬细胞瘤:临床表现、诊断与治疗
J Hypertens. 2006 Dec;24(12):2331-9. doi: 10.1097/01.hjh.0000251887.01885.54.
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Sustentaculoma: report of a case of a distinctive neoplasm of the adrenal medulla.
Am J Surg Pathol. 2006 Feb;30(2):268-73. doi: 10.1097/01.pas.0000178095.07513.38.

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