Department of Endocrinology, Diabetes and Isotope Therapy, Medical University Wroclaw, Wrocław, Poland.
Department of Endocrinology, Metabolism and Internal Medicine, University of Medical Sciences, Poznan, Poland, Poznan, Poland.
Endokrynol Pol. 2019;70(1):2-18. doi: 10.5603/EP.a2018.0093.
Acromegaly is a rare disease caused by excessive production of growth hormone (GH), typically by a pituitary tumour. The diagnosis is usually delayed, and patients frequently develop various complications that cause premature mortality. In patients with hypertension, heart failure, diabetes, and arthropathies that are not age-specific, attention should be paid to signs of acromegaly. Insulin-like growth factor 1 (IGF-1) assay should be used as a screening test whenever acromegaly is suspected. Further diagnostic investigations and treatment should be carried out at specialist centres. First-line treatment involves selective excision of pituitary adenoma using transsphenoidal access. Patients with chances of cure with surgical removal of the pituitary tumour should be referred to centres that have experience in this type of procedure, following pharmacological preparation. Other patients, as well as patients after failed neurosurgical treatment, should first receive chronic treatment with first-generation somatostatin analogues. For second-line treatment, pasireotide, pegvisomant, cabergoline, or combinations thereof should be considered. In every case, acromegaly sequelae require life-long monitoring and active treatment. Current recommendations, being an updated version of the recommendations published in Endokrynologia Polska in 2014, which take into account the Polish situation, should prove useful in the management of patients with acromegaly.
肢端肥大症是一种由生长激素(GH)过度产生引起的罕见疾病,通常由垂体肿瘤引起。该疾病的诊断通常会被延误,患者常发生多种导致过早死亡的并发症。在患有高血压、心力衰竭、糖尿病和非年龄特异性关节病的患者中,应注意肢端肥大症的迹象。只要怀疑患有肢端肥大症,就应使用胰岛素样生长因子 1(IGF-1)测定作为筛查试验。进一步的诊断检查和治疗应在专科中心进行。一线治疗包括经蝶窦入路选择性切除垂体腺瘤。有机会通过手术切除垂体瘤治愈的患者,应在接受药物准备后转至有此类手术经验的中心。其他患者以及神经外科治疗失败的患者,应首先接受第一代生长抑素类似物的慢性治疗。对于二线治疗,应考虑使用培高利特、培维索孟、卡麦角林或它们的组合。在任何情况下,肢端肥大症的后遗症都需要终生监测和积极治疗。当前的建议是 2014 年在《波兰内分泌学杂志》上发表的建议的更新版本,考虑到了波兰的情况,这应该有助于肢端肥大症患者的管理。