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[I型多发性内分泌腺瘤病——韦尔默综合征]

[Type I multiple endocrine neoplasia--Wermer syndrome].

作者信息

Schmidt C, Schumacher S, Siewert B, Klingmüller D, Vogel J

出版信息

Schweiz Rundsch Med Prax. 1989 Aug 29;78(35):935-40.

PMID:2572044
Abstract

Multiple endocrine neoplastic diseases are genetically determined conditions with particular organ patterns for endocrine tumors. In Type I or Wermer's syndrome the endocrine pancreas, anterior pituitary and parathyroids are involved, insulinoma being the most frequent pancreatic tumor. To facilitate diagnosis, a prolonged oral glucose tolerance test, a fasting test and determination of the glucose-insulin ratio are recommended. Localisation is sought by computer tomography and angiography. A gastrinoma is excluded on the basis of normal gastrin levels in serum and by means of the secretin-provocation-test. Pituitary tumors can be classified more closely with prolactin levels and releasing-hormone tests (LH-RH and TRH). Prolactinoma is the most frequent pituitary tumor and amenable to bromocryptin treatment. If Wermer's syndrome is suspected, primary hyperparathyroidism has to be excluded on the basis of calcium and parathormone levels. Chief cell hyperplasia or multiple adenomas are frequent. Surgical resection is necessary.

摘要

多发性内分泌肿瘤病是由基因决定的疾病,具有内分泌肿瘤的特定器官模式。在I型或韦默综合征中,内分泌胰腺、垂体前叶和甲状旁腺会受累,胰岛素瘤是最常见的胰腺肿瘤。为便于诊断,建议进行延长口服葡萄糖耐量试验、空腹试验及葡萄糖 - 胰岛素比值测定。通过计算机断层扫描和血管造影来寻找肿瘤的定位。根据血清胃泌素水平正常及通过促胰液素激发试验排除胃泌素瘤。垂体肿瘤可通过催乳素水平和释放激素试验(促黄体生成素释放激素和促甲状腺激素释放激素)进行更精确分类。催乳素瘤是最常见的垂体肿瘤,可用溴隐亭治疗。如果怀疑韦默综合征,必须根据钙和甲状旁腺激素水平排除原发性甲状旁腺功能亢进。主细胞增生或多发腺瘤很常见。手术切除是必要的。

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