摘要
目的通过对垂体柄阻断综合征(PSIS)患者临床特点进行分析,提高对该病的认识和诊断水平。方法回顾性分析已确诊的15例PSIS患者的临床表现、实验室检查和影像学特点。结果 PSIS患者15例,其中男12例,女3例。15例均有生长激素缺乏、低促性腺激素性腺功能减退;其中14例合并继发性甲状腺功能减退;其中12例垂体-肾上腺皮质轴功能减退;1例患者有中枢性尿崩症。垂体磁共振增强扫描显示15例患者均存在垂体柄显示不清,垂体前叶体积减小,垂体后叶异位。结论 PSIS的发病率低,男性发病率大于女性,以生长发育迟缓为主要临床表现,伴部分性或完全性腺垂体功能减退。诊断主要依靠磁共振检查,垂体核磁影像多具特征性,应提高临床对PSIS的认识和诊治能力。
Objective To analyze the clinical features of pituitary stalk interruption syndrome（PSIS）. To improve the diagnosis of PSIS. Methods The retrospective analysis was performed on clinical characteristics,laboratory examinations and pituitary MRI diagnosis of 15 cases. Results The patients in this study included 12 males and 3 females. All these 15 patients were lack of growth hormone and gonadotropin hormone. Among them,14 patients presented with hypothyroidism and 12 patients presented with central adrenocortical hypofunction. 1 patient showed central diabetes insipidus. MRI scans of all 15 patients showed a classic triad of anterior pituitary hypoplasia or aplasia,interrupted pituitary stalk and absent or ectopic posterior pituitary. Conclusion PSIS has a male predominance and is rarely familial. Patients with PSIS can be associated with variable endocrine disorders,ranging from isolated growth hormone deficiency（IGHD）to multiple pituitary hormone deficiency（MPHD）. Pituitary MRI scan enable an early recognition and treatment of PSIS.
出处
《中国卫生标准管理》
2016年第10期31-33,共3页
China Health Standard Management
关键词
垂体柄阻断综合征
腺垂体功能减退征
临床诊断
治疗
Pituitary stalk interruption syndrome（PSIS）
Hypopituitarism
Clinical diagnosis
Treatment
