Găloiu S, Poiană C
"C.I. Parhon" National Institute of Endocrinology.
"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
J Med Life. 2015 Oct-Dec;8(4):411-5.
Acromegaly is a rare disease most frequently due to a GH secreting pituitary adenoma. Without an appropriate therapy, life of patients with acromegaly can be shortened with ten years. Pituitary surgery is usually the first line therapy for GH secreting pituitary adenomas. A meta-analysis proved that mortality is much lower in operated patients, even uncured, than the entire group of patients and is similar with the general population in patients with GH<1 μg/ L. For the patients with hypersecreting postoperative remnant tumor, those with low chance of surgical cure or with life-threatening comorbidities, medical therapies are available: somatostatin receptor analogues (SRA), dopamine agonists (DA) and GH receptor antagonists. Studies with >30% utilization of SRAs reported a lower mortality ratio than studies with lower percentages of SRA administration. Although therapy with DA has long been used in patients with acromegaly, there are no studies reporting its effect on mortality, but its efficacy is limited by the low remission rate obtained. The use of conventional external radiotherapy, although with good remission rate in time, was linked with increased mortality, mostly due to cerebrovascular diseases.
Mortality in acromegaly can be reduced to expected levels from general population by using modern therapies either in monotherapy or by using multimodal approaches in experienced centers.
肢端肥大症是一种罕见疾病,最常见的病因是分泌生长激素的垂体腺瘤。若不进行适当治疗,肢端肥大症患者的寿命可能缩短十年。垂体手术通常是分泌生长激素的垂体腺瘤的一线治疗方法。一项荟萃分析证明,接受手术的患者,即使未治愈,其死亡率也远低于整个患者群体,且生长激素水平<1μg/L的患者的死亡率与普通人群相似。对于术后残留肿瘤分泌过多、手术治愈机会低或有危及生命的合并症的患者,有多种药物治疗方法可供选择:生长抑素受体类似物(SRA)、多巴胺激动剂(DA)和生长激素受体拮抗剂。使用SRA比例>30%的研究报告的死亡率低于SRA使用比例较低的研究。虽然DA治疗长期以来一直用于肢端肥大症患者,但尚无研究报告其对死亡率的影响,但其疗效因缓解率低而受限。传统的外照射放疗虽然能及时取得较好的缓解率,但与死亡率增加有关,主要是由于脑血管疾病。
通过在经验丰富的中心采用单一疗法或多模式方法使用现代疗法,肢端肥大症的死亡率可降至普通人群的预期水平。