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意大利临床内分泌学家协会(AME)和意大利 AACE 分会临床实践立场声明:肢端肥大症-第 2 部分:治疗问题。

Italian Association of Clinical Endocrinologists (AME) and Italian AACE Chapter Position Statement for Clinical Practice: Acromegaly - Part 2: Therapeutic Issues.

机构信息

Division of Endocrinology, Niguarda Hospital, Milan, Italy.

Section of Endocrinology and Internal Medicine, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.

出版信息

Endocr Metab Immune Disord Drug Targets. 2020;20(8):1144-1155. doi: 10.2174/1871530320666200129113328.

Abstract

Any newly diagnosed patient should be referred to a multidisciplinary team experienced in the treatment of pituitary adenomas. The therapeutic management of acromegaly always requires a personalized strategy. Normal age-matched IGF-I values are the treatment goal. Transsphenoidal surgery by an expert neurosurgeon is the primary treatment modality for most patients, especially if there are neurological complications. In patients with poor clinical conditions or who refuse surgery, primary medical treatment should be offered, firstly with somatostatin analogs (SSAs). In patients who do not reach hormonal targets with first-generation depot SSAs, a second pharmacological option with pasireotide LAR or pegvisomant (alone or combined with SSA) should be offered. Irradiation could be proposed to patients with surgical remnants who would like to be free from long-term medical therapies or those with persistent disease activity or tumor growth despite surgery or medical therapy. Since the therapeutic tools available enable therapeutic targets to be achieved in most cases, the challenge is to focus more on the quality of life.

摘要

任何新诊断的患者都应转至擅长治疗垂体腺瘤的多学科团队。肢端肥大症的治疗管理始终需要个性化的策略。正常年龄匹配的 IGF-I 值是治疗目标。由专家神经外科医生进行的经蝶窦手术是大多数患者的主要治疗方式,尤其是存在神经并发症的患者。对于临床状况较差或拒绝手术的患者,应首先提供一线药物治疗,即使用生长抑素类似物(SSA)。对于第一代长效 SSA 未达到激素目标的患者,应提供第二代药物选择,即帕瑞肽 LAR 或培维索孟(单独使用或与 SSA 联合使用)。对于希望免除长期药物治疗的手术残留患者,或对于尽管手术或药物治疗后仍存在疾病活动或肿瘤生长的患者,可以提出放疗。由于现有的治疗工具可使大多数情况下达到治疗目标,因此挑战在于更关注生活质量。

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本文引用的文献

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