Okano K, Furukawa Y, Morii H, Fujita T
J Clin Endocrinol Metab. 1982 Aug;55(2):238-43. doi: 10.1210/jcem-55-2-238.
Fourteen patients with pseudohypoparathyroidism, 17 with idiopathic hypoparathyroidism, and 12 with postoperative hypoparathyroidism were treated with vitamin D2, dihydrotachysterol, 1 alpha-hydroxyvitamin D3)1 alpha-OHD3), and 1,25-dihydroxyvitamin D3 for 6-18 months. The optimal maintenance dose or minimum daily dose of 1,25-dihydroxyvitamin D3 to maintain serum calcium at approximately 8.5 mg/100 ml and control all the clinical symptoms was 1.3 +/- 0.16 micrograms/day (mean +/- SE) in pseudohypoparathyroidism, 1.5 +/- 0.18 micrograms/day in idiopathic hypoparathyroidism, and 1.9 +/- 0.50 micrograms/day in postoperative hypoparathyroidism. There was no significant difference in the optimal maintenance dose among the 3 groups. The optimal maintenance dose of 1 alpha-OHD3, however, was 2.0 +/- 0.12 micrograms/day in pseudohypoparathyroidism, significantly lower than that in idiopathic hypoparathyroidism (3.5 +/-0.29 micrograms/day; P less than 0.001) and in postoperative hypoparathyroidism (4.89 +/- 0.54 micrograms/day; P less than 0.001). Significantly lower doses were required in the treatment of idiopathic hypoparathyroidism than in postoperative hypoparathyroidism (P less than 0.05). No significant difference was found in the optimal maintenance dose of dihydrotachysterol and vitamin D2 among the 3 groups. The average pretreatment serum calcium levels and clinical manifestations were indistinguishable among the 3 groups of patients. This suggests that such a difference in the optimal maintenance dose of 1 alpha-OHD3 is ascribed not to the difference in the severity of hypoparathyroidism, but most probably to differences in the pathophysiological processes in pseudohypoparathyroidism and idiopathic or postoperative hypoparathyroidism. The excess parathyroid hormone levels in blood of patients with pseudohypoparathyroidism (and not in other types of hypoparathyroidism) may explain such a difference.
对14例假性甲状旁腺功能减退患者、17例特发性甲状旁腺功能减退患者和12例甲状旁腺功能减退术后患者,使用维生素D2、双氢速甾醇、1α-羟维生素D3(1α-OHD3)和1,25-二羟维生素D3进行了6至18个月的治疗。在假性甲状旁腺功能减退患者中,将血清钙维持在约8.5mg/100ml并控制所有临床症状所需的1,25-二羟维生素D3的最佳维持剂量或最小日剂量为1.3±0.16μg/天(均值±标准误),特发性甲状旁腺功能减退患者为1.5±0.18μg/天,甲状旁腺功能减退术后患者为1.9±0.50μg/天。三组间最佳维持剂量无显著差异。然而,1α-OHD3在假性甲状旁腺功能减退患者中的最佳维持剂量为2.0±0.12μg/天,显著低于特发性甲状旁腺功能减退患者(3.5±0.29μg/天;P<0.001)和甲状旁腺功能减退术后患者(4.89±0.54μg/天;P<0.001)。治疗特发性甲状旁腺功能减退所需的剂量显著低于甲状旁腺功能减退术后患者(P<0.05)。三组间双氢速甾醇和维生素D2的最佳维持剂量未发现显著差异。三组患者治疗前的平均血清钙水平和临床表现无明显差异。这表明1α-OHD3最佳维持剂量的这种差异并非归因于甲状旁腺功能减退严重程度的差异,而很可能是由于假性甲状旁腺功能减退与特发性或甲状旁腺功能减退术后甲状旁腺功能减退在病理生理过程上的差异。假性甲状旁腺功能减退患者血液中甲状旁腺激素水平过高(而其他类型的甲状旁腺功能减退患者则不然)可能解释了这种差异。