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生长激素缺乏合并垂体柄中断13例临床分析

[Clinical analysis of 13 cases with growth hormone deficiency combined with pituitary stalk interruption].

作者信息

Wang Gao-liang, Fu Jun-fen, Wang Chun-lin, Liang Li

机构信息

Department of Endocrinology, Children's Hospital of Zhejiang University School of Medicine, Hangzhou 310003, China.

出版信息

Zhonghua Er Ke Za Zhi. 2010 Apr;48(4):305-7.

Abstract

OBJECTIVE

To analyze the clinical characteristics of the patients with pituitary stalk interruption syndrome (PSIS), and to achieve better comprehension of this disease.

METHOD

Data of 13 patients with PSIS were retrospectively analyzed for the clinical, laboratory and imaging features.

RESULT

All the 13 patients (9 male, 4 female) had the chief complaint of growth retardation, 81.5 - 135.0 cm in body height, which were minus two standard deviations below the average of the normal children of same age and same sex. GH stimulated peak levels were all below 5 microg/L; Among them, one was accompanied by delayed sexual development, one by central diabetes insipidus, one was complicated with central hypothyroidism and one was accompanied by central adrenocortical hypofunction.

CONCLUSION

The most remarkable clinical manifestations of patients with PSIS were growth retardation, partial or complete adenohypophyseal dysfunction. MRI revealed absence of pituitary stalk or anterior pituitary hypoplasia with ectopic posterior pituitary gland.

摘要

目的

分析垂体柄阻断综合征(PSIS)患者的临床特征,以更好地认识该疾病。

方法

回顾性分析13例PSIS患者的临床、实验室及影像学特征。

结果

13例患者(男9例,女4例)均以生长发育迟缓为主诉,身高81.5~135.0cm,低于同年龄、同性别的正常儿童平均身高2个标准差。生长激素刺激峰值均低于5μg/L;其中1例伴有性发育延迟,1例伴有中枢性尿崩症,1例合并中枢性甲状腺功能减退,1例伴有中枢性肾上腺皮质功能减退。

结论

PSIS患者最显著的临床表现为生长发育迟缓、部分或完全性腺垂体功能障碍。磁共振成像显示垂体柄缺如或垂体前叶发育不全伴垂体后叶异位。

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