METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
方法:来自16个国家/地区的56名肢端肥大症专家回顾并讨论了当前的证据,重点是生化测定,诊断标准和成像的作用,病理学,和临床评估;诊断延迟的后果;缓解标准和随访建议;以及评估和监测在定义疾病进展中的价值,选择合适的治疗方法,并最大限度地提高患者的治疗效果。
结果: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>1.3倍的正常年龄上限证实了诊断。在禁食过夜后测量的随机生长激素(GH)可能有助于告知预后,但不是诊断所必需的。对于结果模棱两可的患者,可以重复使用相同的验证测定的IGF-I测量,和口服葡萄糖耐量测试也可能是有用的。虽然生化缓解是治疗结果的主要评估,生化结果应在肢端肥大症的临床背景下进行解释.后续评估应考虑生化评估治疗效果,评估残留/复发腺瘤肿块的影像学研究,以及肢端肥大症的临床体征和症状,其并发症,和合并症。对于生化模棱两可的患者,应考虑转诊到多学科垂体中心,病理学,或诊断时的影像学发现,以及对标准治疗方法反应不足的患者。
结论:共识建议强调了肢端肥大症患者对GH和IGF-I紊乱的新认识,以及专家治疗这种罕见疾病的重要性。