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肢端肥大症、结节性甲状腺肿和无症状性多骨纤维发育不良。McCune-Albright综合征的一种变异型。

Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia. A variant of the McCune-Albright syndrome.

作者信息

Abs R, Beckers A, Van de Vyver F L, De Schepper A, Stevenaert A, Hennen G

机构信息

Department of Endocrinology, University of Antwerp, Wilrijk, Belgium.

出版信息

J Endocrinol Invest. 1990 Sep;13(8):671-5. doi: 10.1007/BF03349592.

Abstract

A 36-year-old woman is reported with a possible variant of the McCune-Albright syndrome. The triad was incomplete because of the absence of skin pigmentation and since the sexual precocity was not evident. The presence of a pituitary mass and the secretory dynamics of growth hormone and prolactin were suggestive of a mammosomatotroph cell adenoma. A toxic multinodular goiter was also associated, but unique was the spontaneous normalization of the thyroid function. Unusual was the silent evolution of the polyostotic fibrous dysplasia, which was only fortuitously discovered during magnetic resonance imaging of the pituitary region. Treatment of the acromegaly with the long-acting somatostatin analogue octreotide resulted in an important inhibition of the GH secretion and in a reduction of the volume of the pituitary adenoma.

摘要

据报道,一名36岁女性可能患有麦库恩-奥尔布赖特综合征的一种变异型。由于没有皮肤色素沉着且性早熟不明显,三联征并不完整。垂体肿块的存在以及生长激素和催乳素的分泌动态提示为乳腺生长激素细胞腺瘤。还伴有毒性多结节性甲状腺肿,但独特的是甲状腺功能自发恢复正常。多骨纤维发育不良的隐匿进展很不寻常,只是在垂体区域的磁共振成像检查中偶然发现。使用长效生长抑素类似物奥曲肽治疗肢端肥大症,导致生长激素分泌受到显著抑制,垂体腺瘤体积缩小。

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