Fleseriu Maria
Department of Medicine, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd., Portland, OR 97239, USA.
Discov Med. 2014 Jun;17(96):329-38.
Acromegaly is a disease characterized by growth hormone (GH) excess originating, in approximately 95% of cases, from a somatotroph pituitary adenoma. Symptomatology and clinical features are due to GH and insulin-like growth factor 1 excess; unfortunately, for most patients diagnosis is delayed by several years. Acromegaly patients' morbidity and mortality are higher than those of the normal population. However, with adequate biochemical control mortality rates can be restored to normal. Tumor size and location, symptoms, comorbidities, and lastly, but not least, patient preference, are all important aspects in treatment decision making, and treatment approach should be individualized. Current therapy includes medical, surgical, and radiation. This review focuses on recent significant developments in medical therapy. There are three major therapeutic drug classes: somatostatin receptor ligands (SRLs), which represent the mainstay of medical therapy, GH receptor blockers, and dopamine agonists. Multi-ligand receptor SRLs such as pasireotide, should increase therapeutic choices for acromegaly patients currently uncontrolled on available SRLs. Furthermore, significant research has been focused in the development of novel delivery modalities (e.g., oral and long acting subcutaneous administration).
肢端肥大症是一种以生长激素(GH)分泌过多为特征的疾病,约95%的病例中,病因是垂体生长激素瘤。其症状和临床特征是由GH和胰岛素样生长因子1分泌过多引起的;不幸的是,大多数患者的诊断会延迟数年。肢端肥大症患者的发病率和死亡率高于正常人群。然而,通过适当的生化控制,死亡率可恢复正常。肿瘤大小和位置、症状、合并症,以及最后但同样重要的患者偏好,都是治疗决策中的重要方面,治疗方法应个体化。目前的治疗方法包括药物治疗、手术治疗和放射治疗。本综述重点关注药物治疗的最新重大进展。有三大类治疗药物:生长抑素受体配体(SRLs),是药物治疗的主要手段;GH受体阻滞剂;以及多巴胺激动剂。多配体受体SRLs,如帕瑞肽,应为目前使用现有SRLs无法控制的肢端肥大症患者增加治疗选择。此外,大量研究集中在新型给药方式(如口服和长效皮下给药)的开发上。