ABNORMALITIES OF THYROID-FUNCTION AND GLUCOSE CONTROL IN SUBJECTS WITH RETT-SYNDROME
ABNORMALITIES OF THYROID-FUNCTION AND GLUCOSE CONTROL IN SUBJECTS WITH RETT-SYNDROME
复制标题
DOI：
10.1159/000184309
复制
发表时间：
1995-06-01
期刊：
影响因子：
--
通讯作者：
BERKOVITZ, GD
中科院分区：
文献类型：
--
作者：
COOKE, DW;NAIDU, S;BERKOVITZ, GD
我们发现雷特综合征患者的甲状腺功能和血糖控制存在细微异常。 Rett 综合征受试者组的平均血清总甲状腺素 (T-4) 浓度（6.9 +/- 1.5 μg/dl，n = 34；p < 0.0001）显着低于成人参考范围（8.5 +/- 1.75 μg/dl，n = 200）。即使对于 17 名未服用抗惊厥药的受试者，这种差异仍然显着（7.6 +/- 1.5 μg/dl；与成人参考相比，p < 0.05）。与年龄调整后的正常人相比，这种差异更为明显，10 名受试者的血清总 T-4 浓度低于正常年龄，其中 17 名受试者中有 3 名未服用抗惊厥药物。血清总 T-4 浓度的降低并非由于通过 3,5,3'-三碘甲状腺原氨酸树脂摄取测量的结合蛋白的变化所致，而是与促甲状腺激素浓度降低相关（1.7 +/- 1.6 mU/l，n = 23 与 2.5 +/- 1.0 mU/l，n = 200；p < 0.01）。对 10 名雷特综合征受试者进行了口服葡萄糖耐量测试。他们的血糖和胰岛素浓度峰值出现延迟。血糖水平在 1 小时和 2 小时时升高（p < 0.05），胰岛素水平在 1、2 和 3 小时时升高（p < 0.05）。两名受试者符合糖耐量受损的标准。
We have identified subtle abnormalities of thyroid function and glucose control in patients with Rett syndrome. The mean serum total thyroxine (T-4) concentration was significantly lower in a group of subjects with Rett syndrome (6.9 +/- 1.5 mu g/dl, n = 34; p < 0.0001) than the adult reference range (8.5 +/- 1.75 mu g/dl, n = 200). This difference remained significant even for the 17 subjects not taking anticonvulsants (7.6 +/- 1.5 mu g/dl; p < 0.05 vs. adult reference). The difference was more marked when compared to age-adjusted normals, with 10 subjects having a serum total T-4 concentration below normal for age including 3 of 17 of the subjects not taking anticonvulsants. This decrease in serum total T-4 concentration was not due to changes in binding proteins as measured by 3,5,3'-triiodothyronine resin uptake, and was associated with a decreased concentration of thyroid-stimulating hormone (1.7 +/- 1.6 mU/l, n = 23 vs. 2.5 +/- 1.0 mU/l, n = 200; p < 0.01). Oral glucose tolerance tests were performed in 10 of the subjects with Rett syndrome. They had a delay in the peak glucose and insulin concentrations. Glucose levels were elevated at 1 and 2 hours (p < 0.05), and insulin levels were elevated at 1, 2, and 3 hours (p < 0.05). Two subjects fulfilled criteria for impaired glucose tolerance.