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特纳综合征中的多重垂体激素缺乏、空蝶鞍和异位神经垂体

Multiple Pituitary Hormone Deficiency, Empty Sella and Ectopic Neurohypophysis in Turner Syndrome.

作者信息

Dutta Deep, Selvan Chitra, Mukhopadhyay Satinath

机构信息

Department of Endocrinology and Metabolism, Institute of Post Graduate Medical Education and Research (IPGMER) and Seth Sukhlal Karnani Memorial (SSKM) Hospital, India. Correspondence to: Dr Deep Dutta, Assistant Professor, Department of Endocrinology, PGIMER and Dr. Ram Manohar Lohia (RML) Hospital, 1 Baba Kharak Singh Marg, New Delhi 110001, India.

出版信息

Indian Pediatr. 2015 Sep;52(9):803-4. doi: 10.1007/s13312-015-0721-z.

Abstract

BACKGROUND

Multiple pituitary hormone deficiency and Turner syndrome have overlapping features in peripubertal girls and is a diagnostic challenge.

CASE CHARACTERISTICS

16-year-old girl having Turner phenotype undergoing evaluation for severe short stature and pubertal arrest.

OBSERVATION

45,X karyotype, and multiple pituitary hormone deficiency with empty sella.

INTERVENTION

Levothyroxine, growth hormone and ethinyl-estradiol replacement resulted in 11 cm height gain with attainment of puberty over 2 years.

MESSAGE

Patients of Turner syndrome with height <3rd percentile (Turner specific charts) warrant additional pathology evaluation.

摘要

背景

多种垂体激素缺乏症与特纳综合征在青春期前女孩中具有重叠特征,是一项诊断挑战。

病例特征

一名16岁具有特纳表型的女孩因严重身材矮小和青春期停滞接受评估。

观察结果

45,X核型,伴有空蝶鞍的多种垂体激素缺乏症。

干预措施

左甲状腺素、生长激素和炔雌醇替代治疗使身高在2年内增加了11厘米,并进入青春期。

要点

身高低于第3百分位(特纳综合征专用图表)的特纳综合征患者需要进行额外的病理评估。

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