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空蝶鞍所致原发性闭经:一种被低估的疾病。

Primary amenorrhea due to empty sella: an underestimated entity.

作者信息

Dange Nitin, Redhu Rakesh, Kawale Juhi, Mahore Amit

机构信息

Kem Hospital, Department of Neurosurgery, Mumbai, India.

出版信息

Turk Neurosurg. 2012;22(4):499-501. doi: 10.5137/1019-5149.JTN.3901-10.0.

DOI:10.5137/1019-5149.JTN.3901-10.0
PMID:22843475
Abstract

We report a 16-year-old girl who had stunted growth and primary amenorrhea presenting with headache and vomiting. MRI of brain showed posterior fossa dermoid cyst with hydrocephalus and empty sella. Growth hormone (GH), Leutinizing hormone (LH) and Follicular stimulating hormone (FSH) were grossly reduced on endocrinological work-up. She underwent suboccipital midline craniectomy and complete excision of dermoid cyst .She had no additional deficit after the surgery. She had menarche six months after the surgery and developed adequate secondary sexual characters, however her height remains subnormal at follow-up after 2 years as compared to age matched controls. We briefly review the radiological features, pathogenesis and outcome of this underestimated entity.

摘要

我们报告一名16岁女孩,她生长发育迟缓且原发性闭经,伴有头痛和呕吐。脑部MRI显示后颅窝皮样囊肿伴脑积水和空蝶鞍。内分泌检查显示生长激素(GH)、促黄体生成素(LH)和促卵泡生成素(FSH)显著降低。她接受了枕下中线颅骨切除术并完整切除了皮样囊肿。术后她没有出现其他功能缺损。术后6个月她月经初潮,并发育出了充分的第二性征,然而与年龄匹配的对照组相比,随访2年后她的身高仍低于正常水平。我们简要回顾了这个被低估的疾病的放射学特征、发病机制和预后。

相似文献

1
Primary amenorrhea due to empty sella: an underestimated entity.空蝶鞍所致原发性闭经:一种被低估的疾病。
Turk Neurosurg. 2012;22(4):499-501. doi: 10.5137/1019-5149.JTN.3901-10.0.
2
Convexity meningioma associated with empty sella presenting as primary amenorrhea.凸面脑膜瘤合并空蝶鞍,表现为原发性闭经。
Neurochirurgia (Stuttg). 1992 Jul;35(4):127-30. doi: 10.1055/s-2008-1052264.
3
[Suprasellar arachnoid cyst associated with precocious puberty: report of an operated case and review of the literature].[鞍上蛛网膜囊肿伴性早熟:1例手术病例报告及文献复习]
No Shinkei Geka. 1992 Oct;20(10):1107-12.
4
Two cases of Kallmann syndrome associated with empty sella.两例与空蝶鞍相关的卡尔曼综合征病例。
Pituitary. 2008;11(1):109-12. doi: 10.1007/s11102-007-0043-9.
5
Progressive "empty sella" syndrome, gonadotropins deficiency and congenital aqueductal stenosis. Case report.进行性“空蝶鞍”综合征、促性腺激素缺乏症与先天性导水管狭窄。病例报告。
Endocrinologie. 1978 Apr-Jun;16(2):153-6.
6
Gamma knife radiosurgery for GH-secreting microadenoma with empty sella.伽玛刀放射外科治疗伴有空蝶鞍的生长激素分泌型微腺瘤。
J Clin Neurosci. 2004 May;11(4):418-21. doi: 10.1016/j.jocn.2003.05.009.
7
Hypothalamic-pituitary evaluation in patients with galactorrhea-amenorrhea and hyperprolactinemia.溢乳-闭经及高催乳素血症患者的下丘脑-垂体评估
Obstet Gynecol. 1980 Jan;55(1):1-7.
8
Transsphenoidal treatment of secondary empty sella syndrome using low field strength intraoperative MRI: case report.
Minim Invasive Neurosurg. 2010 Aug;53(4):198-202. doi: 10.1055/s-0030-1263188. Epub 2010 Dec 3.
9
Primary empty sella: a histologic and immunocytologic study.原发性空蝶鞍:一项组织学和免疫细胞学研究。
Arch Intern Med. 1979 Feb;139(2):248-9.
10
Radiologically atypical congenital posterior fossa dermoid cyst presenting late in life.放射学表现不典型的先天性后颅窝皮样囊肿,于生命晚期出现。
J Clin Neurosci. 2008 Jul;15(7):835-8. doi: 10.1016/j.jocn.2007.03.023. Epub 2008 Jan 30.

引用本文的文献

1
Pituitary Volume in Patients with Primary Empty Sella and Clinical Relevance to Pituitary Hormone Secretion: A Retrospective Single Center Study.原发性空蝶鞍患者的垂体体积及其与垂体激素分泌的临床相关性:一项回顾性单中心研究。
Curr Med Imaging. 2021;17(8):1018-1024. doi: 10.2174/1573405617666210525111218.
2
Rare empty sella syndrome found after postoperative hypotension and respiratory failure: A case report.术后低血压和呼吸衰竭后发现罕见的空蝶鞍综合征:一例报告。
World J Clin Cases. 2019 Mar 6;7(5):663-667. doi: 10.12998/wjcc.v7.i5.663.