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[家族性垂体肿瘤]

[Familial pituitary tumors].

作者信息

Yoshimoto K, Saito S

机构信息

Otsuka Department of Clinical and Molecular Nutrition, University of Tokushima.

出版信息

Nihon Rinsho. 1995 Nov;53(11):2691-6.

PMID:8538028
Abstract

Familial pituitary tumors are relatively rare. Most commonly, they occur as a part of multiple endocrine neoplasia type 1 (MEN 1). However, familial pituitary adenomas unrelated MEN 1 (familial pituitary adenomas) are extremely rare. In review of MEN 1 in Japan, 60% of the patients with MEN 1 had pituitary tumors. Only 45 cases of familial pituitary adenomas have been reported from 20 families. In our review of familial pituitary adenomas, 30 (67%) of 45 reported cases are acromegaly or gigantism. This incidence is much higher than 28% in MEN 1 patients with pituitary tumors. Allelic deletions at 11q13 were identified in MEN 1 associated pituitary adenomas and familial pituitary adenomas in two gigantism brothers.

摘要

家族性垂体瘤相对罕见。最常见的是,它们作为多发性内分泌腺瘤1型(MEN 1)的一部分出现。然而,与MEN 1无关的家族性垂体腺瘤(家族性垂体腺瘤)极为罕见。在对日本MEN 1的回顾中,60%的MEN 1患者患有垂体瘤。仅报道了来自20个家族的45例家族性垂体腺瘤。在我们对家族性垂体腺瘤的回顾中,45例报告病例中有30例(67%)为肢端肥大症或巨人症。这一发病率远高于患有垂体瘤的MEN 1患者中的28%。在两个患巨人症的兄弟的MEN 1相关垂体腺瘤和家族性垂体腺瘤中发现了11q13的等位基因缺失。

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