中国全科医学2016，Vol.19Issue(20)：2486-2489,2492,5.DOI:10.3969/j.issn.1007-9572.2016.20.026
垂体柄中断综合征21例临床特征分析
Clinical Features Analysis of 21 Patients With Pituitary Stalk Interruption Syndrome
尹倩倩 1左春林 1胡红琳 1邓大同 1钱银锋 1王长江 1章秋1
作者信息
- 1. 230022 安徽省合肥市，安徽医科大学第一附属医院内分泌科，影像科
- 折叠
摘要
Abstract
Objective To investigate the clinical features of pituitary stalk interruption syndrome(PSIS),to improve the understanding of the disease,and to ensure early detection,early diagnosis and early treatment. Methods PSIS patients who received treatment in the First Affiliated Hospital of Anhui Medical University from June 2010 to September 2015 were enrolled,and a retrospective analysis of patients′ general data,examination results of laboratory and iconography and treatment condition was made in this study. Results Among the 21 PSIS patients,20 cases came to see a doctor due to short stature with gonadal dysgenesis,1 case because of pure gonadal dysgenesis. 21 cases were lack of growth hormone,of which 19 cases were absolute growth hormone deficiency;13 cases were gonad hypofunction of low gonadotropin;13 cases were central adrenocortical hypofunction;8 cases were central hypothyroidism;and 2 cases were central diabetesinsipidus. Small volume of pituitary gland of patients,vague or absent pituitary stalk,and ectopic posterior pituitary were presented by pituitary magnetic resonance imaging (MRI)inspection. Replacement therapy of growth therapy was given to the 21 growth hormone deficiency patients,regular follow- up was made and supplement of sex hormones was not provided until their stature reached the expected target or finished growing. Glucocorticoid replacement therapy was given to the 13 patients with adrenocortical hypofunction,and the 8 patients with hypothyroidism were treated with oral levothyroxine sodium. Conclusion PSIS,a disease of low incidence,is primarily featured as short stature clinically,which is usually combined with multiple anterior pituitary hormonoprivia syndrome. The diagnosis of PSIS relies on MRI,and timely hormone replacement therapy should be administered after definitive diagnosis.关键词
垂体柄中断综合征/性腺发育不全/诊断/治疗Key words
Pituitary stalk interruption syndrome/Gonadal dysgenesis/Diagnosis/Therapy分类
医药卫生引用本文复制引用
尹倩倩,左春林,胡红琳,邓大同,钱银锋,王长江,章秋..垂体柄中断综合征21例临床特征分析[J].中国全科医学,2016,19(20):2486-2489,2492,5.
