垂体柄阻断综合征的临床诊治

Clinical Diagnosis and Treatment of Pituitary Stalk Interruption Syndrome

垂体柄阻断综合征(PSIS)是一种临床罕见的先天性多垂体激素缺乏性疾病。近年来关于PSIS的病例报道及磁共振对该病的影像学诊断价值的文献日趋增多,但是对该病系统阐述的文献很少。现就PSIS这一疾病发病机制的遗传和基因方面的特点、选择何种激素以及何时行替代治疗等方面进行综述,以指导临床对该病的全面认识及规范处理。

Pituitary stalk interruption syndrome（PSIS）is a rare kind of congenital multiple pituitary hormone deficiency disease.In recent years,the reported cases of PSIS and the valuable literatures related to nuclear magnetic resonance imaging（MRI）diagnosis of this disease are increasing,while the literature of systemic description of the cases of PSIS is rare.Here is to make a review on the genetic mechanism of the pathogenesis,hormone treatment choice and timing of substitute treatment of PSIS,in order to guide the comprehensive understanding and regular treatment in clinical.