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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 summarize the clinical features of pituitary stalk disruption syndrome (PSIS). Methods The clinical features, laboratory findings and imaging features of 114 PSIS patients admitted to the Department of Endocrinology of Chinese PLA General Hospital were retrospectively analyzed. Results Of the 114 patients, 102 (89.4%) had an average age of (21.1 ± 6.1) years old, 91 (91.9%) had abnormal birth position, 89 (71.8% ) Years, secondary sexual characteristics generally poorly developed. Growth hormone deficiency, sexual dysfunction, adrenal insufficiency, hypothyroidism were 100.0%, 94.0%, 84.2%, 74.6%, hyperprolactinemia was 28.1%. There were three or more pituitary hormone abnormalities in 105 patients (92.1%). There were no significant differences in height (t = 0.297, P = 0.634), penis length (t = 1.205, P = 0.882), testis volume (U = 99.000) in 53 male breech production patients and 5 male patients with head position production , P = 0.348), the peak value of excitability test for growth hormone (U = 89.000, P = 0.186), the peak value of excitability test for adrenotropin (U = 131.000, P = 0.967) 0.582), thyroid stimulating hormone (U = 82.000, P = 0.162) and anterior pituitary height (t = 1.676, P = 0.107). Conclusion The clinical manifestations, symptoms, and hormone deficits of PSIS patients in this group are serious. There is no significant difference in the severity of PSIS in this group of patients with different birth patterns.