Gershengorn M C, Weintraub B D
J Clin Invest. 1975 Sep;56(3):633-42. doi: 10.1172/JCI108133.
An 18-yr-old woman with clinical and laboratory features of hyperthyroidism had persistently elevated serum levels of immunoreative thyrotropin (TSH). During 11 yr of follow-up there had been no evidence of a pituitary tumor. After thyrotropin-releasing hormone (TRH), there was a marked increase in TSH and secondarily in triiodothyronine (T3), the latter observation confirming the biologic activity of the TSH. Exogenous T3 raised serum T3 and several measurements of peripheral thyroid hormone effect, while decreasing serum TSH, thyroxine (T4), and thyroidal radioiodine uptake. After T3, the TRH-stimulated TSH response was decreased but was still inappropriate for the elevated serum T3 levels. Dexamethasone reduced serum TSH but did not inhibit TRH stimulation of TSH. Propylthiouracil reduced serum T4 and T3 and raised TSH. This patient represents a new syndrome of TSH-induced hyperthyroidism, differing from previous reports in the absence of an obvious pituitary tumor and in the responsiveness of the TSH to TRH stimulation and thyroid hormone suppression. This syndrome appears to be caused by a selective, partial resistance of the pituitary to the action of thyroid hormone. This case is also compared with previous reports in the literature of patients with elevated serum levels of immunoreactive TSH in the presence of elevated total and free thyroid hormones. A classification of these cases, termed "inappropriate secretion of TSH," is proposed.
一名18岁女性具有甲状腺功能亢进的临床和实验室特征,其血清免疫反应性促甲状腺激素(TSH)水平持续升高。在11年的随访中,未发现垂体肿瘤的证据。注射促甲状腺激素释放激素(TRH)后,TSH显著升高,继而三碘甲状腺原氨酸(T3)升高,后一观察结果证实了TSH的生物活性。外源性T3使血清T3升高,并多次测量外周甲状腺激素效应,同时降低血清TSH、甲状腺素(T4)和甲状腺放射性碘摄取。给予T3后,TRH刺激的TSH反应降低,但仍与升高的血清T3水平不相称。地塞米松降低血清TSH,但不抑制TRH对TSH的刺激。丙硫氧嘧啶降低血清T4和T3并升高TSH。该患者代表了一种新的TSH诱导的甲状腺功能亢进综合征,与先前报道不同之处在于没有明显的垂体肿瘤,以及TSH对TRH刺激和甲状腺激素抑制的反应性。该综合征似乎是由垂体对甲状腺激素作用的选择性部分抵抗引起的。本文还将该病例与文献中先前报道的血清免疫反应性TSH水平升高而总甲状腺激素和游离甲状腺激素水平也升高的患者进行了比较。提出了对这些病例的分类,称为“TSH不适当分泌”。