中枢性甲亢2例及文献复习
Two cases of central hyperthyroidism report and literature review

卞毅,胡蜀红.中枢性甲亢2例及文献复习[J].内科急危重症杂志,2015,21(5):

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英文关键词:central hyperthyroidism, TSH adenoma, thyroid hormone resistance syndrome,

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作者	单位	E-mail
卞毅	华中科技大学同济医学院附属同济医院急诊科	89609301@qq.com
胡蜀红	华中科技大学同济医学院附属同济医院内分泌科	hushuho2645@163.com

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中文摘要:

目的: 探讨中枢性甲状腺功能亢进症的诊断和治疗。方法: 回顾在我院住院的2例中枢性甲亢患者的诊治经过并结合文献进行分析。结果: 两例患者均有甲状腺肿大和甲亢症状,血甲状腺激素(fT4,fT3)和促甲状腺激素(TSH)都轻度升高。其中1例甲亢症状经2年口服抗甲状腺药物和2次放射性131I治疗症状亦无缓解,垂体MRI示垂体侵袭性大腺瘤,且伴生长激素分泌过多依据,经蝶行垂体瘤切除术,瘤体组织免疫组化TSH和 GH染色阳性,术后血FT3, FT4 和TSH较术前明显下降,诊断垂体TSH腺瘤。另外1例垂体MRI未见异常,血TSH可被地塞米松和溴隐亭抑制,甲状腺激素受体β亚单位基因(THRB)检测未发现突变。诊断为选择性垂体甲状腺激素抵抗综合征(PRTH), 选择非特异性对症治疗。结论: 对有甲亢临床表现、血fT4和fT3升高而 TSH不降低(明显有别于Graves 病等原发性甲亢)的甲亢患者,应考虑中枢性甲状腺功能亢进症,其病因可能为垂体TSH腺瘤或PRTH。进一步进行垂体MRI,TSH抑制或兴奋试验,TSHα亚单位检测,THRB基因突变检测,等检查有助于垂体TSH腺瘤和PRTH的诊断以及两者的鉴别诊断,以利选择正确的治疗方案。

英文摘要:

Objective :To discuss about diagnosis and treatment of central hyperthyroidism. Methods: Two male patients of central hyperthyroidism were retrospectively analysed and related literature were reviewed. Results: Both patients were found to have goiter and hyperthyroidism ,blood concentraion of thyroid hormone(fT4 and fT3) were elevated , meanwhile thyrotropin(TSH) concentration were not suppressed.One of them had persistent hyperthyroidism even after antithyroid drugs treatment for 2 years and two times radioactive 131I treatment. Pituitary MRI suggested a invasive macroadenoma, hormone investigation found also growth hormone hypersecretion. These patient underwent transsphenoidal adenoma resection,immunohistochemical stain of the adenoma preparate found TSH( ) angd GH( ). These Patient was diagnosed with pituitary TSH-adenoma. The other patient had no lesion in pituitary MRI, blood TSH levels could be suppressed by dexamethasone and bromocriptine, no mutation was found in 9-12 exons of thyroid receptor β coding gene(THRB),these patient was diagnosed with selective pituitary resistance to thyroid hormone(PRTH), and received nonspecifically symptomatic treatment. Conclusions:For patients with hypertyrodism demonstrating elevated thyroid hormone( fT4 and fT3) while not suppressed TSH should central hyperthyroidism suspected, the causes may be pituitary TSH adenoma or PRTH, further pituitary MRI, TSH inhibiting or exciting tests ,TSHα subunit determination and THRB gene mutation test could help to diagnosis and differential diagnosis of these two disease, and help to selest appropriate treatment