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3173 例来自列日肢端肥大症调查(LAS)数据库的患者的诊断时肢端肥大症。

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database.

机构信息

Department of EndocrinologyCHU de Liège, University of Liège, Belgium.

Clinical Centre of Endocrinology and GerontologyMedical University, Sofia, Bulgaria.

出版信息

Endocr Relat Cancer. 2017 Oct;24(10):505-518. doi: 10.1530/ERC-17-0253. Epub 2017 Jul 21.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9e67/5574208/7471910ffda0/erc-24-505-g001.jpg

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