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Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

机译：尼尔森综合症：临床表现，病理生理学和治疗策略的综述

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摘要

Nelson's syndrome is a rare clinical manifestation that occurs in 8%-47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.
机译：尼尔森氏综合征是一种罕见的临床表现，其发生在8％-47％的患者中，是双侧肾上腺切除术的并发症，该过程用于控制库欣病患者的皮质醇过多症。此病于1958年由唐·尼尔森（Don Nelson）博士首次描述，此后已与色素沉着过多，肾上腺皮质激素分泌过多和皮质营养腺瘤的临床三联症有关。即使这样，在过去的几年中，对纳尔逊氏综合征的诊断标准和治疗方法还没有进行足够的研究。尼尔森氏综合症的主要治疗方法是经蝶窦手术。在许多情况下已被用作手术的辅助治疗的其他独立疗法包括放射疗法，放射手术和药物疗法。双侧肾上腺切除术时的预防性放疗可以预防尼尔森综合征（保护作用）。最有前途的药物是替莫唑胺，奥曲肽和帕瑞肽，但这些药物通常在经蝶窦手术后给药。在鼠模型中，罗格列酮已显示出一定的功效，但在人体研究中尚未发现这些结果。在本文中，作者回顾了纳尔逊综合征的临床表现，病理生理，诊断标准和多峰治疗策略的疗效。

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