促甲状腺激素不适当分泌综合征61例病例总结  

Syndrome of inappropriate secretion of TSH ： Differential diagnosis in 61 patients

作　　者：叶蕾[1] 韩如来 姜晓华[1] 苏颋为[1] 姜蕾[1] 赵萸[1] 王曙[1] 王卫庆[1] 宁光[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院内分泌代谢病科,上海市内分泌代谢病临床医学中心,上海市内分泌代谢病研究所,200025

出　　处：《中华内分泌代谢杂志》2015年第11期925-931,共7页Chinese Journal of Endocrinology and Metabolism

基　　金：上海市科委重点项目（11DJ1400200）;上海市启明星跟踪计划（13QH1402000）

摘　　要：目的 分析促甲状腺激素（TSH）瘤与甲状腺激素抵抗综合征（RTH）的鉴别诊断要点.方法 回顾分析2004年1月至2014年3月瑞金医院61例临床表现为FT3、FT4升高,TSH正常或升高患者的临床资料.结果 垂体TSH瘤27例,RTH 20例,不能明确诊断14例.主要就诊原因为心慌心悸（54％）.TSH瘤与RTH在性别组成、血清甲状腺激素水平、血清性激素结合球蛋白水平差异无统计学意义.TSH瘤平均诊断年龄较晚[（45.59±11.32对31.80± 18.20）岁,P=0.012],起病年龄较晚[（42.16± 12.53对23.30±15.77）岁,P＜0.01],疾病病程较短[（3.44 ±4.55对8.50±9.95）年,P=0.049].垂体MRI发现5例RTH患者存在占位病变.对其中40例促甲状腺激素不适当分泌综合征患者行促甲状腺素释放激素（TRH）兴奋试验,TSH平均升高（5.34±4.11）倍,其中TSH瘤患者平均升高（2.35±1.61）倍,RTH患者升高（9.48±2.94）倍（P＜0.01）;45例行善宁抑制试验,TSH平均抑制率为66.73％,TSH瘤患者平均抑制率77.02％,RTH患者为52.33％ （P＜0.01）.12例RTH患者存在甲状腺激素受体β亚基突变.结论 TSH瘤较RTH患者起病较晚且病程相对较短;因为RTH患者可以合并垂体微腺瘤,故垂体MRI不能有效鉴别两种疾病;TRH兴奋试验可以有效鉴别两种疾病.Objective To analyze the diagnostic approaches to differentiate thyrotropin （TSH）-secreting pituitary adenomas （TSHoma） and resistance to thyroid hormone （RTH）.Methods This was a retrospective single center study.All patients with insuppressible TSH despite elevated free-T4 and free-T3 levels from 2004 to 2014 were enrolled.Results Twenty-seven patients were diagnosed as TSHoma, 20 as RTH, and 14 remained undetermined.Palpitation was observed in 54% patients as the main symptom.There was no significant difference in gender, serum levels of sex hormone-binding globulin, and free thyroid hormones between patients with TSHoma and RTH.The ages of patients at diagnosis and disease onset were older in TSHoma patients than RTH patients [（45.59 ± 11.32 vs 31.80 ± 11.32） years, P =0.012;（42.16 ± 12.53 vs 23.30 ± 15.77） years, P〈0.01].The course of disease was shorter in TSHoma patients than RTH patients [（3.44 ± 4.55 vs 8.50 ± 9.95） years, P =0.049].Five RTH patients showed pituitary adenoma by MRI.TSHoma patients showed blunt TSH response to thyrotropin hormone releasing hormone （TRH） stimulation while RTH patients showed normal TRH stimulation [（2.35 ± 1.61 vs 9.48 ± 2.94） fold, P 〈 0.01].TSHoma patients showed significantly suppressed serum TSH by somatostatin analogs as compared with RTH patients [（77.02 ± 13.43） % vs （52.33 ± 15.02） %, P〈0.01].Twelve RTH patients had thyroid hormone receptor beta （TR[β） mutation.Conclusion A later age of disease onset and shorter disease course suggested TSHoma other than RTH.TRH stimulating test was the most accurate test to distinguish RTH and TSHoma as compared with pituitary MRI, somatostatin suppression test, and TRβ mutation analysis.

关 键 词：促甲状腺激素瘤 甲状腺激素抵抗综合征 

分 类 号：R584[医药卫生—内分泌]