Clinical characteristics and efficacy of acromegaly treatment in the Udmurt Republic

G. Nurullina, G. I. Akhmadullina, I. S. Maslova, Alexandra R. Nikolaeva
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Abstract

Background: Acromegaly is a severe neuroendocrine disorder caused by chronic overproduction of growth hormone and insulin-like growth factor 1 and associated with a variety of clinical manifestations, debilitating complications and progressive disability, if no biochemical control has been achieved. The Russian registry of hypothalamic-pituitary tumors allows for evaluation of the epidemiological, demographic and clinical characteristics of patients with acromegaly, as well as of the effectiveness of various treatment methods. Aim: To identify epidemiological and clinical characteristics and treatment efficacy for acromegaly in the patients in the Udmurt Republic. Materials and methods: We performed a cross-sectional study based on the registry of hypothalamic-pituitary tumors of the Udmurt Republic as per June 2022. It contains data from 77 patients with acromegaly. The study included 59 (76.62%) patients with the information sufficient for evaluation of the disease stage and its complications. Results: According to the registry of hypothalamic-pituitary tumors, the prevalence of acromegaly in the Udmurt Republic was 6.71 cases per 100,000 of the population. Patients with acromegaly in Udmurt Republic had a high prevalence of arthralgia, abnormally large feet and hands, weakness/fatigue, snoring / sleep apnea, increased sweating, dizziness, peripheral edema, obesity, menstrual cycle abnormalities, diabetes mellitus, nodular goiter, pathological fractures and hypopituitarism. Neurosurgery has been performed in 74.57% (n = 44) of the patients, with a remission of acromegaly achieved in 18 (40.9%). Medical treatment was given to 25/59 (42.37%) of the patients, with 22 of them being treated with 1st generation long-acting somatostatin analogues. Radiation therapy has been administered to 13 patients (22.03%). Biochemical control was achieved in 40/59 (67.8%) of patients with acromegaly, related to neurosurgery in most patients, as well as to the use of a growth hormone receptor antagonist and/or combined medical treatment. Conclusion: The prevalence of acromegaly in the Udmurt Republic is consistent with international data. Clinical characteristics of acromegaly generally correspond to the classic disease course, although a number of symptoms are more commonly found in the Udmurt Republic patients than in the Russian Federation registry; this might be related to the quality of the registry management. The effectiveness of treatment for acromegaly in Udmurt Republic is comparable to the international data.
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乌德穆尔特共和国肢端肥大症治疗的临床特点和疗效
背景:肢端肥大症是一种严重的神经内分泌疾病,由生长激素和胰岛素样生长因子1的慢性过量产生引起,如果没有生化控制,可伴有多种临床表现、衰弱并发症和进行性残疾。俄罗斯下丘脑-垂体肿瘤登记处允许评估肢端肥大症患者的流行病学、人口学和临床特征,以及各种治疗方法的有效性。目的:了解乌德穆尔特共和国肢端肥大症患者的流行病学、临床特点及治疗效果。材料和方法:我们根据2022年6月乌德穆尔特共和国下丘脑垂体肿瘤的登记进行了一项横断面研究。它包含了77例肢端肥大症患者的数据。本研究纳入59例(76.62%)患者,资料足以评估疾病分期及其并发症。结果:根据下丘脑-垂体肿瘤登记,乌德穆尔特共和国肢端肥大症的患病率为每10万人6.71例。乌德穆尔特共和国肢端肥大症患者普遍存在关节痛、异常大的脚和手、虚弱/疲劳、打鼾/睡眠呼吸暂停、出汗增多、头晕、周围水肿、肥胖、月经周期异常、糖尿病、结节性甲状腺肿、病理性骨折和垂体功能减退。74.57% (n = 44)的患者接受了神经外科手术,其中18例(40.9%)肢端肥大症得到缓解。59例患者中有25例(42.37%)接受了药物治疗,其中22例接受了第一代长效生长抑素类似物治疗。13例患者(22.03%)接受放射治疗。59例肢端肥大症患者中有40例(67.8%)实现了生化控制,这与大多数患者的神经外科手术以及使用生长激素受体拮抗剂和/或联合药物治疗有关。结论:乌德穆尔特共和国肢端肥大症的患病率与国际数据一致。肢端肥大症的临床特征一般符合典型病程,尽管乌德穆尔特共和国患者的一些症状比俄罗斯联邦登记的患者更常见;这可能与注册中心管理的质量有关。乌德穆尔特共和国肢端肥大症的治疗效果与国际数据相当。
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