Livadas D P, Sofroniadou K, Souvatzoglou A, Boukis M, Siafaka L, Koutras D A
Clin Endocrinol (Oxf). 1984 Apr;20(4):435-43. doi: 10.1111/j.1365-2265.1984.tb03439.x.
Thyroid and pituitary function tests using hypothalamic releasing factors were performed in seven patients with thalassaemia and secondary haemosiderosis and in a control group of seven healthy subjects. The TSH level in the thalassaemic patients (18.07 +/- 1.10 microU/ml) was higher than in the controls (1.01 +/- 0.14 microU/ml, P less than 0.001). After TRH administration the TSH values increased less than in controls. Serum thyroxine and FT41 values were lower in the group of patients with thalassaemia (76.7 +/- 7.8 nmol/l and 19.3 +/- 2.2) compared to the controls (116.1 +/- 6.9 nmol/l, P less than 0.005 and 38.6 +/- 3.6, P less than 0.001). The basal prolactin values did not differ significantly between the two groups, but after TRH administration the increment was significantly lower in thalassaemics than in controls (P less than 0.005). The basal LH values were lower in the thalassaemic patients (1.37 +/- 0.24 ng/ml) than in the controls (3.23 +/- 0.50 ng/ml) and did not increase significantly after LHRH administration. The FSH values were also lower in the thalassaemic group (0.46 +/- 0.15 ng/ml) compared to the controls (2.06 +/- 0.08 ng/ml, P less than 0.001), and increased only slightly after LHRH administration. We conclude that in thalassaemia pituitary deficiency exists, mostly of gonadotrophs, but possibly also for the thyrotrophs and the lactotrophs. Latent primary hypothyroidism has also been found in the thalassaemic group. The functional abnormalities found in both endocrine glands are best explained as a consequence of coexisting haemosiderosis.
对7例地中海贫血合并继发性含铁血黄素沉着症患者及7名健康对照者进行了使用下丘脑释放因子的甲状腺和垂体功能测试。地中海贫血患者的促甲状腺激素(TSH)水平(18.07±1.10微单位/毫升)高于对照组(1.01±0.14微单位/毫升,P<0.001)。注射促甲状腺激素释放激素(TRH)后,TSH值的升高幅度低于对照组。与对照组相比,地中海贫血患者组的血清甲状腺素和游离甲状腺素指数(FT4I)值较低(分别为76.7±7.8纳摩尔/升和19.3±2.2)(对照组分别为116.1±6.9纳摩尔/升,P<0.005;38.6±3.6,P<0.001)。两组的基础催乳素值无显著差异,但注射TRH后,地中海贫血患者的升高幅度显著低于对照组(P<0.005)。地中海贫血患者的基础促黄体生成素(LH)值(1.37±0.24纳克/毫升)低于对照组(3.23±0.50纳克/毫升),注射促黄体生成素释放激素(LHRH)后无显著升高。地中海贫血组的促卵泡激素(FSH)值(也低于对照组(0.46±0.15纳克/毫升)(对照组为2.06±0.08纳克/毫升,P<0.001),注射LHRH后仅略有升高。我们得出结论,在地中海贫血中存在垂体功能不足,主要是促性腺激素细胞功能不足,但也可能涉及促甲状腺激素细胞和催乳激素细胞。在地中海贫血组中还发现了潜在的原发性甲状腺功能减退。这两个内分泌腺中发现的功能异常最好解释为是共存的含铁血黄素沉着症的结果。