垂体柄中断综合征临床特点分析(1)
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【摘要】 目的 分析、总结垂体柄中断综合征(PSIS)的临床特点及内分泌功能收变,提高对该病的认识和诊断水平。方法 回顾性分析我院7例PSIS患者的临床表现、内分泌实验室检查和影像学特点。结果 7例患者中男5例,女2例,就诊年龄5~29岁,2例有难产史,起病时身高118~152 cm,6例表现为生长发育迟缓,1例表现为口干、多饮、多尿及低比重尿。实验室检查结果提示:5例患者有生长激素缺乏、低促性腺激素性腺功能减退和垂体-肾上腺皮质轴功能减退,其中3例合并继发性甲状腺功能减退;1例患者仅表现为中枢性尿崩症;1例患者目前垂体前后叶内分泌功能均正常。磁共振检查显示所有患者垂体前叶体积变小,垂体柄显示不清,垂体正常后叶高信号消失,6例患者可见异位的垂体后叶,1例伴有ChiariI畸形及C1~4锥体水平脊髓空洞。结论 PSIS发病率低,诊断主要依靠磁共振检查,可出现垂体前叶及后叶功能障碍,病情早期,垂体柄阻断不完全时,亦可无垂体功能障碍。
【关键词】垂体柄中断综合征;生长发育迟缓;临床特征
Cliinical analysis of 7cases of pituitary stalk interruption syndrome
LIANG Zhi-li,YAO Bin,WANG Li-chun,et al.Department of Endocrinology,The First Affiliated Hospital ofSun Yat-sen University,Guangzhou 510080,China
【Abstract】 Objective To improve the accuracy of the clinical diagnosis of pituitary stalk interruption syndrome(PSIS) and improve its knowledge and diagnosis.Methods Seven cases(five males and two females)were retrospectively reviewed in terms of clinical manifestations,laboratory findings and imaging features.Results Age of onset ranges from 5 to 29 years,height during onset from 118 to 152 cm.Six cases had a history of growth retardation,one had thirst,polydipsia and low urine specific gravity.As for laboratory findings,5 cases had deficiency of growth hormone,gonadotropin hormone and adrenal corticotrophin hormone,among which 3 presented with secondary hypothyroidism,1 with central diabetes insipidus,and 1 with normal pituitary function.MRI findings of reduced volume of the anterior pituitary,vague pituitary stalk,absence of hypersignality of the posterior pituitary,were seen in all cases.Ectopic posterior pituitary was revealed in 6 cases.One had concomitant ChiariI malformation,another with cervical 1-4 level syringomyelia.Conclusion The incidence of PSIS is low.Clinical manifestation is associated with anterior and/or posterior pituitary dysfunction,which could be cryptic during early phase of disease,when obstruction of the pituitary stalk is subtotal.Diagnosis is made primarily based on MRI.
【Key words】
Pituitary stalk interruption syndrome; Growth hormone deficiency; Clinical characteristics
垂体柄中断综合征(pituitary stalk interruption syndrome,PSIS)是指垂体柄缺如, 垂体后叶异位。下丘脑分泌的激素不能经过垂体柄运送到垂体后叶,无法通过垂体门脉系统作用于垂体前叶所致的一系列临床症侯群[1] ......
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