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目的分析垂体柄中断综合征(pituitary stalk interruption syndrome,PSIS)的误诊情况及其原因。方法回顾性分析2004-2014年解放军总医院确诊的110例PSIS患者的临床资料,分析曾被误诊PSIS的病因和特征。结果 110例PSIS初次就诊时误诊率95.7%(96/110),中位误诊时间6.0年;误诊的病种依次为特发性单纯生长激素缺乏37例(38.5%)、垂体前叶功能减退13例(13.5%)、生长发育迟缓12例(12.5%)、垂体发育不良9例(9.4%)、特发性低促性腺功能减退症8例(8.3%)、原发性甲状腺功能减退症8例(8.3%)、其他疾病9例(9.4%)。误诊病例出生时臀位或足先露68例(70.8%),有难产史者27例(28.1%);生长激素完全性缺乏96例(100%),促性腺激素缺乏66例(68.8%)、促肾上腺皮质激素缺乏59例(61.5%),高泌乳素血症30例(31.3%),促甲状腺激素缺乏1例(1.0%),≥3种垂体前叶激素缺乏57例(59.4%)。结论 PSIS误诊率高,对于生长激素等垂体前叶激素缺乏者,尤其出生时臀位或足先露者,应及早全面评估垂体功能并行垂体MRI检查,避免误诊。
Objective To analyze the misdiagnosis and its causes of pituitary stalk interruption syndrome (PSIS). Methods The clinical data of 110 patients with PSIS diagnosed in PLA General Hospital from 2004 to 2014 were analyzed retrospectively to analyze the etiology and characteristics of PSIS who had been misdiagnosed. Results The initial misdiagnosis rate of PSIS was 95.7% (96/110) and the median time of misdiagnosis was 6.0 years. The misdiagnosed diseases were as follows: 37 cases (38.5%) with idiopathic growth hormone deficiency, 13 cases with anterior pituitary hypofunction (13.5%), growth retardation in 12 cases (12.5%), pituitary dysplasia in 9 cases (9.4%), idiopathic hypogonadism in 8 cases (8.3%), primary hypothyroidism in 8 Cases (8.3%), other diseases in 9 cases (9.4%). There were 68 cases (70.8%) with breech or foot deformity at birth, 27 cases (28.1%) with labor history, 96 cases (100%) with complete lack of growth hormone and 66 cases (68.8%) with gonadotropin deficiency (61.5%), hyperprolactinemia (31.3%), thyrotropin deficiency in 1 case (1.0%), and ≥3 hypopituitarism in 57 cases (59.4%), . Conclusions The high misdiagnosis rate of PSIS should not be misdiagnosed as to evaluate the pituitary function parallel pituitary MRI examination as early as possible for the anterior pituitary hormone such as growth hormone and other breech or foot predators.