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1型多发性内分泌腺瘤病

[Multiple endocrine neoplasia type 1].

作者信息

Salmela Pasi I

机构信息

OYS, medisiininen tulosalue, sisätautien ja keuhkosairauksien vastuualue.

出版信息

Duodecim. 2012;128(22):2345-54.

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is a complex multisystem disease characterized by the combined occurrence of endocrine tumours of the parathyroid glands, anterior pituitary gland and adrenal glands and the neuroendocrine tumours (NET) of duodenum, pancreas, thymus and bronchus. Malignancy occurs commonly (up to 30 %) and malignant NETs are important causes of the MEN1-related morbidity and mortality. Regular clinical, biochemical and radiologic screening for the syndrome-related tumours are the basis of the life-long surveillance. Early detection and adequate management of these tumours reduce risk of death and morbidity.

摘要

1型多发性内分泌腺瘤病(MEN1)是一种复杂的多系统疾病,其特征是甲状旁腺、垂体前叶和肾上腺的内分泌肿瘤以及十二指肠、胰腺、胸腺和支气管的神经内分泌肿瘤(NET)合并出现。恶性肿瘤很常见(高达30%),恶性NET是MEN1相关发病和死亡的重要原因。对综合征相关肿瘤进行定期的临床、生化和放射学筛查是终身监测的基础。早期发现并妥善处理这些肿瘤可降低死亡和发病风险。

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