San Raffaele Vita-Salute University and IRCCS Hospital, Milan, Italy.
Leiden University Medical Center, Leiden, The Netherlands.
Pituitary. 2024 Feb;27(1):7-22. doi: 10.1007/s11102-023-01360-1. Epub 2023 Nov 3.
The 14th Acromegaly Consensus Conference was convened to consider biochemical criteria for acromegaly diagnosis and evaluation of therapeutic efficacy.
Fifty-six acromegaly experts from 16 countries reviewed and discussed current evidence focused on biochemical assays; criteria for diagnosis and the role of imaging, pathology, and clinical assessments; consequences of diagnostic delay; criteria for remission and recommendations for follow up; and the value of assessment and monitoring in defining disease progression, selecting appropriate treatments, and maximizing patient outcomes.
In a patient with typical acromegaly features, insulin-like growth factor (IGF)-I > 1.3 times the upper limit of normal for age confirms the diagnosis. Random growth hormone (GH) measured after overnight fasting may be useful for informing prognosis, but is not required for diagnosis. For patients with equivocal results, IGF-I measurements using the same validated assay can be repeated, and oral glucose tolerance testing might also be useful. Although biochemical remission is the primary assessment of treatment outcome, biochemical findings should be interpreted within the clinical context of acromegaly. Follow up assessments should consider biochemical evaluation of treatment effectiveness, imaging studies evaluating residual/recurrent adenoma mass, and clinical signs and symptoms of acromegaly, its complications, and comorbidities. Referral to a multidisciplinary pituitary center should be considered for patients with equivocal biochemical, pathology, or imaging findings at diagnosis, and for patients insufficiently responsive to standard treatment approaches.
Consensus recommendations highlight new understandings of disordered GH and IGF-I in patients with acromegaly and the importance of expert management for this rare disease.
第 14 届肢端肥大症共识会议的目的是讨论肢端肥大症诊断的生化标准和治疗效果评估。
来自 16 个国家的 56 位肢端肥大症专家对目前针对生化检测的证据进行了回顾和讨论;讨论了诊断标准以及影像学、病理学和临床评估的作用;诊断延迟的后果;缓解标准和随访建议;评估和监测在确定疾病进展、选择适当治疗方法和最大化患者结局方面的价值。
在具有典型肢端肥大症特征的患者中,胰岛素样生长因子(IGF)-I>正常年龄上限的 1.3 倍可确诊该病。隔夜禁食后随机测量生长激素(GH)可能有助于预测预后,但并非诊断所必需。对于结果不确定的患者,可以重复使用相同的经过验证的检测方法测量 IGF-I,口服葡萄糖耐量试验也可能有用。虽然生化缓解是治疗效果的主要评估方法,但应在肢端肥大症的临床背景下解读生化结果。随访评估应考虑评估治疗效果的生化评估、评估残余/复发性腺瘤肿块的影像学研究,以及肢端肥大症、其并发症和合并症的临床体征和症状。对于诊断时生化、病理或影像学检查结果不确定的患者,以及对标准治疗方法反应不足的患者,应考虑转诊至多学科垂体中心。
共识建议强调了对肢端肥大症患者中异常 GH 和 IGF-I 的新认识,以及对这种罕见疾病进行专家管理的重要性。