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[下丘脑错构瘤所致性早熟]

[Precocious puberty due to hypothalamic hamartoma].

作者信息

Luba Magdalena, Bossowski Artur, Kulikowska Justyna, Urban Mirosława, Szarras-Czapnik Maria, Rutkowski Robert, Parol Tomasz, Gasiorek Tomasz, Sawicka Beata, Skrzydło Marcin

机构信息

Koło Naukowe przy ll Klinice Chorób Dzieci UM w Białymstoku.

出版信息

Pediatr Endocrinol Diabetes Metab. 2008;14(4):257-61.

PMID:19239796
Abstract

INTRODUCTION

Central precocious puberty is usually idiopathic. Appearance of the precocious puberty symptoms in early childhood or pre-school period indicate that also it could be caused by organic disorder of the central nervous system. The aim of this work is to present the case of the 4-year-old girl, diagnosed with precocious puberty.

THE CASE REPORT

The first clinical symptoms of precocious puberty such as increased growth rate and breast enlargement were observed when the girl was 4 years old. The height (above the 97 centile) and weight (90-97 centile) were measured during the physical examination. The advancement of sexual features was determined as follows: thelarche III degrees , pubarche II degrees , axillarche II degrees . The LHRH test used in this differential diagnosis revealed the pubertal level of gonadotropins, when plasma levels of dehydroepiandrosterone, prolactin, thyroid-stimulating hormone and alpha-fetoprotein levels were correct. The advanced bone age was 8 years and 10 months, while the height age was 7 years. The final diagnosis was based on MRI scan. The patient is currently treated with an analog of gonadoliberine (Diphereline). In conclusion, we aspired to notice that the pharmacological treatment of hypothalamic hamartoma may be safe and effective. Suppression of puberty to the normal time of pubescence gives a child the chance to reduce health discomforts as well as further emotional and social problems.

摘要

引言

中枢性性早熟通常是特发性的。在幼儿期或学龄前出现性早熟症状也表明可能是由中枢神经系统的器质性疾病引起的。本文旨在介绍一名4岁诊断为性早熟女孩的病例。

病例报告

该女孩4岁时观察到性早熟的最初临床症状,如生长速度加快和乳房增大。体格检查时测量了身高(高于第97百分位数)和体重(第90 - 97百分位数)。性征发育进展情况如下:乳房发育III度,阴毛发育II度,腋毛发育II度。该鉴别诊断中使用的促性腺激素释放激素(LHRH)试验显示促性腺激素达到青春期水平,而脱氢表雄酮、催乳素、促甲状腺激素和甲胎蛋白的血浆水平正常。骨龄提前至8岁10个月,而身高年龄为7岁。最终诊断基于磁共振成像(MRI)扫描。患者目前正在接受促性腺激素释放激素类似物(达必佳)治疗。总之,我们希望注意到下丘脑错构瘤的药物治疗可能是安全有效的。将青春期抑制到正常青春期时间,能让孩子减少健康不适以及进一步的情感和社会问题。

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