PSEUDOHYPOPARATHYROIDISM - DEFECTIVE EXCRETION OF 3',5'-AMP IN RESPONSE TO PARATHYROID HORMONE
PSEUDOHYPOPARATHYROIDISM - DEFECTIVE EXCRETION OF 3',5'-AMP IN RESPONSE TO PARATHYROID HORMONE
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DOI：
10.1172/jci106149
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发表时间：
1969-01-01
影响因子：
15.9
通讯作者：
AURBACH, GD
中科院分区：
文献类型：
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作者：
CHASE, LR;MELSON, GL;AURBACH, GD
对正常人、假性甲状旁腺功能减退症、特发性甲状旁腺功能减退症、手术性甲状旁腺功能减退症和假性甲状旁腺功能减退症患者在基础状态下和输注纯化甲状旁腺激素15分钟后的尿环腺苷酸（3′，5 ′-AMP）排泄进行了检测。低钙症患者的基础核苷酸排泄量低于正常值，假性甲状旁腺功能减退症患者的基础核苷酸排泄量高于正常值。甲状旁腺激素引起除假性甲状旁腺功能减退症患者外的所有受试者的3′，5 ′-AMP排泄显著增加; 9例假性甲状旁腺功能减退症患者对甲状旁腺激素无反应，4例反应明显不足。放射免疫分析表明，假性甲状旁腺功能减退症患者血浆中甲状旁腺激素循环量增加，当血清钙升高至12 mg/100 ml以上时，甲状旁腺激素检测不到。通过诱导高钙血症抑制甲状旁腺激素分泌并不能改变对外源性激素的反应不足。结果表明：（a）假性甲状旁腺功能减退症患者的甲状旁腺激素循环浓度异常高，其分泌对钙的生理控制反应正常;（B）测定尿中3′，5 ′-AMP对输注纯化甲状旁腺激素的反应是诊断假性甲状旁腺功能减退症的一个准确而敏感的指标;和（c）所述疾病的代谢缺陷可由骨和肾中甲状旁腺素敏感性腺苷酸环化酶的缺乏或缺陷形式来解释。
Urinary excretion of cyclic adenosine 3′,5′-monophosphate (3′,5′-AMP) was tested in normal subjects and patients with pseudohypoparathyroidism, idiopathic hypoparathyroidism, surgical hypoparathyroidism, and pseudopseudohypoparathyroidism under basal conditions and after a 15 min infusion of purified parathyroid hormone. Basal excretion of the nucleotide was less than normal in the patients with hypocalcemic disorders and greater than normal in pseudopseudohypoparathyroidism. Parathyroid hormone caused a marked increase in excretion of 3′,5′-AMP in all subjects except those with pseudohypoparathyroidism; nine patients with this disorder did not respond to the hormone and four showed a markedly deficient response. Radioimmunoassay showed that parathyroid hormone circulated in increased amounts in plasma from patients with pseudohypoparathyroidism and became undetectable when serum calcium was increased above 12 mg/100 ml. Suppression of parathyroid hormone secretion by induction of hypercalcemia did not alter the deficient response to exogenous hormone. The results indicate that: (a) parathyroid hormone circulates in abnormally high concentrations in pseudohypoparathyroidism and secretion of the hormone responds normally to physiological control by calcium; (b) testing urinary excretion of 3′,5′-AMP in response to infusion of purified parathyroid hormone appears to be an accurate and sensitive index for establishing the diagnosis of pseudohypoparathyroidism; and (c) the metabolic defect of the disorder can be accounted for by a lack of or defective form of parathyroid hormone-sensitive adenyl cyclase in bone and kidney.