Department of EndocrinologyCHU de Liège, University of Liège, Belgium.
Clinical Centre of Endocrinology and GerontologyMedical University, Sofia, Bulgaria.
Endocr Relat Cancer. 2017 Oct;24(10):505-518. doi: 10.1530/ERC-17-0253. Epub 2017 Jul 21.
Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The , a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; < 0.001). The median delay from first symptoms to diagnosis was 2 years longer in females ( = 0.015). Ages at diagnosis and first symptoms increased significantly over time ( < 0.001). Tumors were larger in males than females ( < 0.001); tumor size and invasion were inversely related to patient age ( < 0.001). Random GH at diagnosis correlated with nadir GH levels during OGTT ( < 0.001). GH was inversely related to age in both sexes ( < 0.001). Diabetes mellitus was present in 27.5%, hypertension in 28.8%, sleep apnea syndrome in 25.5% and cardiac hypertrophy in 15.5%. Serious cardiovascular outcomes like stroke, heart failure and myocardial infarction were present in <5% at diagnosis. Erythrocyte levels were increased and correlated with IGF-1 values. Thyroid nodules were frequent (34.0%); 820 patients had colonoscopy at diagnosis and 13% had polyps. Osteoporosis was present at diagnosis in 12.3% and 0.6-4.4% had experienced a fracture. In conclusion, this study of >3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis.
肢端肥大症是一种由慢性生长激素(GH)分泌过多引起的罕见疾病。虽然诊断和治疗方法已经有所进步,但关于肢端肥大症特征随时间变化的趋势的信息很少。该数据库是一个关系数据库,旨在评估来自十个国家的 3173 例肢端肥大症患者在多个治疗中心诊断时和长期随访期间的特征。以下是诊断时的结果。研究人群包括来自十个国家的 3173 例肢端肥大症患者;54.5%为女性。男性在诊断时比女性明显年轻(43.5 岁比 46.4 岁;<0.001)。女性从首发症状到诊断的中位延迟时间长 2 年(=0.015)。诊断年龄和首发症状随时间显著增加(<0.001)。男性肿瘤大于女性(<0.001);肿瘤大小和侵袭性与患者年龄呈反比(<0.001)。诊断时的随机 GH 与 OGTT 时的 GH 最低点相关(<0.001)。GH 在两性中均与年龄呈反比(<0.001)。糖尿病占 27.5%,高血压占 28.8%,睡眠呼吸暂停综合征占 25.5%,心脏肥大占 15.5%。诊断时<5%存在严重心血管事件,如中风、心力衰竭和心肌梗死。红细胞水平升高,与 IGF-1 值相关。甲状腺结节常见(34.0%);820 例患者在诊断时进行了结肠镜检查,13%的患者有息肉。诊断时骨质疏松症占 12.3%,0.6-4.4%的患者发生过骨折。总之,这项对超过 3100 例患者的研究是最大的国际肢端肥大症数据库,显示了诊断时肢端肥大症特征的临床相关趋势。