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一种使用人甲状旁腺激素合成1-38片段对甲状旁腺功能减退症和I型假性甲状旁腺功能减退症进行的简化诊断测试。

A simplified diagnostic test in hypoparathyroidism and pseudohypoparathyroidism type I with synthetic 1-38 fragment of human parathyroid hormone.

作者信息

Kruse K, Kracht U

机构信息

Universitäts-Kinderklinik, Würzburg, Federal Republic of Germany.

出版信息

Eur J Pediatr. 1987 Jul;146(4):373-7. doi: 10.1007/BF00444941.

Abstract

Bovine parathyroid extract of E. Lilly (Indianapolis), which has been used to differentiate pseudohypoparathyroidism (PHP) and hypoparathyroidism (HP) is no longer available. We therefore evaluated the usefulness of the synthetic 1-38 fragment of human parathyroid hormone (1-38 hPTH), the biologically active part of the intact hormone, in a simplified modification of the traditional Ellsworth-Howard test. 1-38 hPTH was slowly injected over 2 min at a dose of 0.5 micrograms/kg body weight in healthy children and adults (n = 7), as well as in children with HP (n = 4) and PHP (n = 4). In the controls PTH administration induced a significant rise of plasma cAMP (at least 146 nmol/l), urine cAMP and serum prolactin, as well as a decrease of the tubular phosphate reabsorption. The respective responses were similar in the children with HP but markedly impaired in the patients with PHP. In four healthy adults a second PTH administration at an interval of 3 h induced a comparable response of cAMP and prolactin, indicating that these PTH effects are reproducible and not impaired by the first injection. The two PTH administrations caused a significant but variable rise of serum 1,25-dihydroxy-vitamin D in all four adults. In conclusion 1-38 hPTH has effects on the kidney and anterior pituitary similar to purified parathyroid extracts but has the advantage of being chemically and biologically defined and well tolerated. Since timed urine collections may be unreliable in young children, the plasma cAMP measurement at 5 and 10 min after the PTH injection is a good substitute for the traditional Ellsworth-Howard test, peak values over 100 nmol/l being regarded as a normal response.

摘要

曾用于鉴别假性甲状旁腺功能减退症(PHP)和甲状旁腺功能减退症(HP)的礼来公司(印第安纳波利斯)的牛甲状旁腺提取物已不再供应。因此,我们在对传统的埃尔斯沃思 - 霍华德试验进行简化改进后,评估了人甲状旁腺激素的合成1 - 38片段(1 - 38 hPTH)(完整激素的生物活性部分)的效用。在健康儿童和成人(n = 7)以及患有HP(n = 4)和PHP(n = 4)的儿童中,以0.5微克/千克体重的剂量在2分钟内缓慢注射1 - 38 hPTH。在对照组中,给予PTH导致血浆cAMP(至少146纳摩尔/升)、尿cAMP和血清催乳素显著升高,以及肾小管对磷酸盐的重吸收减少。患有HP的儿童的相应反应相似,但患有PHP的患者的反应明显受损。在4名健康成年人中,间隔3小时再次给予PTH诱导了cAMP和催乳素的类似反应,表明这些PTH效应是可重复的,并且不受首次注射的影响。两次给予PTH在所有4名成年人中均导致血清1,25 - 二羟基维生素D显著但可变的升高。总之,1 - 38 hPTH对肾脏和垂体前叶的作用类似于纯化的甲状旁腺提取物,但具有化学和生物学性质明确且耐受性良好的优点。由于幼儿定时收集尿液可能不可靠,在注射PTH后5分钟和10分钟测量血浆cAMP是传统埃尔斯沃思 - 霍华德试验的良好替代方法,峰值超过100纳摩尔/升被视为正常反应。

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