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伴有垂体腺瘤的家族性肢端肥大症。三名患病同胞的报告。

Familial acromegaly with pituitary adenoma. Report of three affected siblings.

作者信息

Abbassioun K, Fatourehchi V, Amirjamshidi A, Meibodi N A

出版信息

J Neurosurg. 1986 Mar;64(3):510-2. doi: 10.3171/jns.1986.64.3.0510.

Abstract

The authors report the cases of three brothers with pituitary adenomas who had classical findings of acromegaly and gigantism. Two had irreducibly elevated growth hormone (GH) values and underwent transsphenoidal microsurgical extirpation of their tumors. The third acromegalic brother had a normal GH value and evidence of panhypopituitarism; he had a small intrasellar tumor and a partially empty sella. The pattern of inheritance was probably autosomal recessive. A review of literature indicated that familial incidence of isolated acromegaly with pituitary adenomas is rare.

摘要

作者报告了三兄弟患垂体腺瘤的病例,他们均有肢端肥大症和巨人症的典型表现。其中两人生长激素(GH)值持续升高,接受了经蝶窦显微手术切除肿瘤。第三个肢端肥大症兄弟的GH值正常,但有全垂体功能减退的证据;他有一个小的鞍内肿瘤和部分空蝶鞍。遗传模式可能为常染色体隐性遗传。文献回顾表明,孤立性肢端肥大症伴垂体腺瘤的家族发病率很低。

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