114例垂体柄中断综合征临床分析  

Clinical Features of Pituitary Stalk Interruption Syndrome in 114 Cases

作　　者：韩白玉[1,2] 张倩[1,3] 李乐乐[1] 郭清华[1] 王成芷[1] 藏丽[1] 金楠[1] 陈芳[1] 赵玲[1] 崔佳[1] 谷秀莲[1] 马芳玲[1] 张赛春[1] 母义明[1] 窦京涛[1] HAN Bai-yu ZHANG Qian LI Le-le GUO Qing-hua WANG Cheng-zhi ZANG Li JIN Nan CHEN Fang ZHAO Ling CUI Jia GU Xiu-lian MA Fang-ling ZHANG Sai-chun MU Yi-ming DOU Jing-tao(Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China Department of Endocrinology, the 264th Hospital of PLA, Taiyuan 030001, China Department of Endocrinology, PLA Army General Hospital, Beijing 100700, China)

机构地区：[1]中国人民解放军总医院内分泌科,北京100853 [2]中国人民解放军264医院内分泌科,太原030001 [3]中国人民解放军陆军总医院内分泌科,北京100700

出　　处：《中国医学科学院学报》2016年第5期534-538,共5页Acta Academiae Medicinae Sinicae

基　　金：国家自然科学基金(81370871)~~

摘　　要：目的总结垂体柄中断综合征(PSIS)的临床特征。方法回顾性分析中国人民解放军总医院内分泌科收治的114例PSIS患者的临床特征、实验室检查、影像学特征。结果 114例患者中,男102例(89.4%),平均年龄(21.1±6.1)岁;91例(91.9%)为异常产位生产,89例(71.8%)身材矮小,骨龄延迟(6.1±5.1)年,第二性征普遍发育不良。生长激素缺乏、性功能低下、肾上腺功能低下、甲状腺功能低下的比例分别为100.0%、94.0%、84.2%、74.6%,高泌乳素血症比例为28.1%。105例患者(92.1%)存在3种以上垂体激素异常。53例男性臀位生产患者与5例男性头位生产患者比较,两组在身高(t=0.297,P=0.634)、阴茎牵长(t=1.205,P=0.882)、睾丸容积(U=99.000,P=0.348)、兴奋试验生长激素峰值(U=89.000,P=0.186)、兴奋试验促肾上腺激素峰值(U=131.000,P=0.967)、兴奋试验黄体生成素峰值(U=98.500,P=0.582)、促甲状腺素值(U=82.000,P=0.162)和垂体前叶高度(t=1.676,P=0.107)方面差异均无统计学意义。结论本组PSIS患者的临床表现、症状、激素缺乏程度严重,出生方式不同的患者病情严重程度无明显差异。Objective To analyze the clinical characteristics of pituitary stalk interruption syndrome（ PSIS）.Methods The clinical data including clinical manifestations,laboratory tests,and imaging findingsof 114 PSIS patients in our hospital were retrospectively analyzed.Results Of these 114 PSIS patients,102 cases（ 89.4%） were male.The average age was 21.1 ± 6.1 years.A history of breech delivery was documented in91 cases（ 91.9%）.Short stature was found in 89 cases（ 71.8%） and bone age delayed（ 6.1 ± 5.1） years.Secondary sex characteristics were poor or undeveloped in most patients.The prevalence of deficiencies in growth hormone,gonadotropins,corticotropin, and thyrotropin were 100.0%,94.0%,84.2%, and 74.6%, respectively.Hyperprolactinemia was found in 28.1% of patients.Three or more pituitary hormone abnormalities were found in 105 cases（ 92.1%）.Compared with the 5 cases with history of cephalic delivery,no difference were found in the aspects of height（ t = 0.297,P = 0.634）,penile length（ t = 1.205,P = 0.882）,testicular volume（ U = 99.000,P = 0.348）,growth hormone peak（ U = 89.000,P = 0.186）,adrenocorticotropic hormone peak（ U = 131.000,P = 0.967）,luteinizing hormone peak（ U = 98.500, P = 0.582）, thyroidstimulating hormone（ U = 82.000,P = 0.162）,and the height of anterior pituitary（ t = 1.676,P = 0.107） in the 53 cases with history of breech delivery.Conclusions The clinical manifestations,symptoms,hormone deficiencies were severe in our series.The condition severities were not remarkably different in patients with different delivery ways.

关 键 词：垂体柄中断综合征 临床特征 垂体功能减退 

分 类 号：R584[医药卫生—内分泌]