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[韦纳-莫里森综合征:内分泌性霍乱还是血管活性肠肽瘤?(作者译)]

[The Verner-Morrison syndrome: endocrine cholera or vipoma? (author's transl)].

作者信息

Charleux H

出版信息

Nouv Presse Med. 1982 Mar 6;11(11):859-62.

PMID:6280131
Abstract

This syndrome, also known under the initials W.D.H.A., is due to a single or multiple pancreatic tumour or to micropolyadenomatosis consisting of non-beta islet cells. Malignancy is found in about two-thirds of the cases. The other endocrine glands are rarely involved. The syndrome is more frequent in women than in men. It is characterized by liquid diarrhoea, marked hypokalaemia and absence of gastric hyperacidity. The tumour is mainly diagnosed by echotomography, computerized tomography and arteriography. It can also be located by staged collections of blood along the portal system for hormonal assays. The nature of the tumour can only be ascertained by demonstrating the presence of the responsible hormone, usually the "vaso-intestinal peptide". Treatment is primarily surgical. Adjuvant treatments include streptozotocine and embolization by superselective catherization in cases of hepatic matastases. The prognosis is sombre since in spite of the various treatments cure can only be achieved in 50% of the patients.

摘要

这种综合征,也以首字母缩写W.D.H.A.为人所知,是由单个或多个胰腺肿瘤或由非β胰岛细胞组成的微小多腺瘤病引起的。约三分之二的病例存在恶性肿瘤。其他内分泌腺很少受累。该综合征在女性中比在男性中更常见。其特征为水样腹泻、明显的低钾血症以及无胃酸过多。肿瘤主要通过超声断层扫描、计算机断层扫描和动脉造影进行诊断。也可通过沿门静脉系统分阶段采集血液进行激素检测来定位肿瘤。肿瘤的性质只能通过证明存在相关激素,通常是“血管活性肠肽”来确定。治疗主要是手术治疗。辅助治疗包括链脲佐菌素,对于肝转移病例,可通过超选择性导管插入术进行栓塞。预后不佳,因为尽管进行了各种治疗,但只有50%的患者能够治愈。

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