Demonstration of a lack of change in serum 1 alpha,25-dihydroxyvitamin D in response to parathyroid extract in pseudohypoparathyroidism.
Demonstration of a lack of change in serum 1 alpha,25-dihydroxyvitamin D in response to parathyroid extract in pseudohypoparathyroidism.
复制标题
证明假性甲状旁腺功能减退症患者对甲状旁腺提取物的反应缺乏血清 1α,25-二羟基维生素 D 的变化。
DOI：
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发表时间：
1980
影响因子：
15.9
通讯作者：
S. Epstein
中科院分区：
文献类型：
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作者：
P. Lambert;B. Hollis;N. Bell;S. Epstein
本研究比较了甲状旁腺提取物（PTE）对正常人血清、尿钙（Ca）、尿磷（P）、血清25-羟维生素D（25-OHD）、血清24，25-二羟维生素D（24，25-OHD）（24，25（OH）2D），血清1 α，25-二羟维生素D（1 α，25（OH）2D）和尿环AMP在两个正常人，2例甲状旁腺功能减退症（HP）患者和6例假性甲状旁腺功能减退症（PHP）患者中，其中一些人正在接受维生素D的次优治疗。研究了两名PHP患者，同时使用1 α，25-（OH）2D 3进行长期治疗。PTE前，1例患者血清1 α，25（OH）2D处于正常下限，其他5例患者异常低。这些个体均未接受1 α，25（OH）2D 3治疗。给予维生素D的患者血清25-OHD和24，25（OH）2D升高或处于正常上限，而其他患者则正常。PTE使正常人和HP患者血清P降低，血清1 α，25（OH）2D、血和尿Ca、尿P和尿cAMP升高。在个别研究中，血清1 α，25（OH）2D和血清Ca的变化在PTE之前，期间和之后平行发生。相比之下，PTE对PHP患者的影响很小。在正常人和HP患者血清1 α，25（OH）2D之间存在高度显著的正相关，而在PHP患者中只有1例存在显著相关。在接受1 α，25（OH）2D 3长期治疗的2例PHP患者中，观察到PTE引起的血清Ca升高。在这些个体中，PTE仅导致血清1 α，25（OH）2D轻微增加。在任何受试者或患者中，PTE均未改变血清25-OHD和24，25（OH）2D。结果提供证据表明，HP和PHP的低钙血症部分源于低循环1 α，25-（OH）2D，并表明PHP患者PTE时血清1 α，25（OH）2D缺乏变化与肾腺苷酸环化酶和磷酸尿反应受损有关。这些和以前的结果支持的想法，减少肾生产的1 α，25（OH）2D，因为甲状旁腺素敏感的腺苷酸环化酶系统的缺陷，可能是一个促成因素的发病机制中的异常钙代谢PHP。
Studies were carried out to compare the effects of parathyroid extract (PTE) on serum and urinary calcium (Ca) and phosphorus (P), serum 25-hydroxyvitamin D (25-OHD), serum 24,25-dihydroxyvitamin D (24,25(OH)2D), serum 1 alpha,25-dihydroxyvitamin D (1 alpha,25(OH)2D), and urinary cyclic AMP in two normal subjects, two patients with hypoparathyroidism (HP) and six patients with pseudohypoparathyroidism (PHP), some of whom were on suboptimal treatment with vitamin D. Two of the patients with PHP were studied while on long-term treatment with 1 alpha,25-(OH)2D3. Before PTE, serum 1 alpha, 25(OH)2D was at the lower limit of normal in one patient and was abnormally low in the other five patients. None of these individuals was on treatment with 1 alpha,25(OH)2D3. Serum 25-OHD and 24,25(OH)2D were either increased or at the upper limit of normal in the patients given vitamin D and were normal in the other patients. PTE lowered the serum P and increased the serum 1 alpha,25(OH)2D, serum and urinary Ca, urinary P, and urinary cyclic AMP in the normal subjects and patients with HP. In individual studies, changes in serum 1 alpha,25(OH)2D and serum Ca occurred in parallel before, during, and after PTE. In contrast, PTE had very little effect in the patients with PHP. Whereas there were highly significant positive correlations between serum 1 alpha,25(OH)2D in each of the normal subjects and patients with HP, there were significant correlations in only one of the patients with PHP. An increase in serum Ca in response to PTE was observed in one of the two patients with PHP who were on long-term treatment with 1 alpha,25(OH)2D3. In these individuals, PTE produced only slight increases in serum 1 alpha,25(OH)2D. Serum 25-OHD and 24,25(OH)2D were not changed by PTE in any of the subjects or patients. The results provide evidence that hypocalcemia in HP and PHP arises in part from low circulating 1 alpha,25-(OH)2D, and indicate that the lack of change in serum 1 alpha,25(OH)2D with PTE in patients with PHP is related to impaired renal adenylate cyclase and phosphaturic responses. These and previous results support the idea that diminished renal production of 1 alpha,25(OH)2D, because of a defect in the parathyroid hormone-responsive adenylate cyclase system, may be a contributing factor in the pathogenesis of the abnormal calcium metabolism in PHP.