Konno N
Endocrinol Jpn. 1977 Aug;23(4):313-7.
The response in serum thyrotropin (TSH) to synthetic thyrotropin-releasing hormone (TRH) as well as serum free thyroxine index (FT4I) and free triiodothyronine index (FT3I) was investigated in six patients with familial thyroxine-binding-globulin (TBG) deficiency. The total serum thyroxine (T4) and triiodothyronine (T3) concentrations were significantly decreased, compared with those of normal subjects (3.4 +/- 0.9 microgram/dl, mean +/- SD. vs. 9.0 +/- 1.5 microgram/dl, p less than 0.01 and 87 +/- 27 ng/dl vs. 153 +/- 37 ng/dl, p less than 0.01, respectively). FT4I was lower than the normal range in all but one (5.3 +/- 1.5 vs. 8.9 +/- 1.6, p less than 0.01), whereas FT3I was all in the normal range and of no significant difference from the normal control (132 +/- 22 vs. 148 +/- 25). Serum TSH concentrations in TBG deficiency were all in the normal range (1.0-4.2 muU/ml) and the maximum TSH increments following TRH 500 microgram iv were 8.9 +/- 2.0 muU/ml and of no significant difference from the normal control (10.2 +/- 4.5 muU/ml). These results indicate that the euthyroid state in familial TBG deficiency is more clearly defined by TRH-test and the normal response to TRH in familial TBG deficiency is presumably under the control of the serum free T3 level rather than the serum free T4 level.
对6例家族性甲状腺素结合球蛋白（TBG）缺乏症患者，研究了血清促甲状腺激素（TSH）对合成促甲状腺激素释放激素（TRH）的反应以及血清游离甲状腺素指数（FT4I）和游离三碘甲状腺原氨酸指数（FT3I）。与正常受试者相比，血清总甲状腺素（T4）和三碘甲状腺原氨酸（T3）浓度显著降低（分别为3.4±0.9μg/dl，均值±标准差，与9.0±1.5μg/dl相比，p<0.01；87±27ng/dl与153±37ng/dl相比，p<0.01）。除1例患者外，其余患者的FT4I均低于正常范围（5.3±1.5与8.9±1.6相比，p<0.01），而FT3I均在正常范围内，与正常对照组无显著差异（132±22与148±25）。TBG缺乏症患者的血清TSH浓度均在正常范围内（1.0 - 4.2μU/ml），静脉注射500μg TRH后TSH的最大增加值为8.9±2.0μU/ml，与正常对照组无显著差异（10.2±4.5μU/ml）。这些结果表明，家族性TBG缺乏症的甲状腺功能正常状态通过TRH试验能更清楚地界定，家族性TBG缺乏症对TRH的正常反应可能受血清游离T3水平而非血清游离T4水平的控制。
