Yamaoka K, Seino Y, Ishida M, Ishii T, Shimotsuji T, Tanaka Y, Kurose H, Matsuda S, Satomura K, Yabuuchi H
J Clin Endocrinol Metab. 1981 Nov;53(5):1096-100. doi: 10.1210/jcem-53-5-1096.
The effect of dibutyryl cAMP on plasma concentrations of 1,25-dihydroxyvitamin D [1,25-(OH)2D] was studied in two patients with pseudohypoparathyroidism (PHP) type I, five normal adults, and three normal children as controls. In normal adults, plasma 1,25-(OH)2D tended to increase 3 h after the infusion of 2.5 mg/kg dbcAMP and was significantly increased after 6 h. In normal children, plasma 1,25-(OH)2D increased 3 h after the infusion, then gradually decreased; in the patients with PHP type I, it increased greatly from 6.8 and 11.2 pg/ml to 264.6 and 128.2 pg/ml 3 h after the infusion. These results suggest that the renal mechanism for the response to parathyroid hormone is intact distal to the renal adenylate cyclase in patients with PHP type I.
在两名Ⅰ型假性甲状旁腺功能减退症(PHP)患者、五名正常成年人及三名正常儿童作为对照的情况下,研究了二丁酰环磷腺苷(dbcAMP)对血浆1,25 - 二羟基维生素D[1,25-(OH)₂D]浓度的影响。在正常成年人中,静脉输注2.5mg/kg dbcAMP后3小时,血浆1,25-(OH)₂D趋于升高,6小时后显著升高。在正常儿童中,输注后3小时血浆1,25-(OH)₂D升高,随后逐渐下降;在Ⅰ型PHP患者中,输注后3小时,其从6.8和11.2pg/ml大幅升至264.6和128.2pg/ml。这些结果表明,Ⅰ型PHP患者肾脏中对甲状旁腺激素反应的机制在肾腺苷酸环化酶远端是完整的。