Silverberg A B, Mennes P A, Cryer P E
Am J Med. 1978 Feb;64(2):231-5. doi: 10.1016/0002-9343(78)90050-5.
In five patients with Bartter's syndrome, mean (+/-SE) plasma norepinephrine concentrations increased from 324 +/- 75 pg/ml with the patients in the supine position to 550 +/- 100 pg/ml, 753 +/- 104 pg/ml and 808 +/- 116 pg/ml after 2,5 and 10 minutes, respectively, in the standing position, levels significantly higher than normal. Plasma epinephrine concentrations were indistinguishable from normal. One patient was shown to resistant the pressor (but not the metabolic) effects of intravenously administered norepinephrine prior to treatment with reversion to normal pressor responsiveness during indomethacin administration. Similarly, that patient's exaggerated endogenous norepinephrine response to standing (10 minute plasma value of 1,110 pg/ml) reverted to normal (10 minute value of 462 pg/ml) during indomethacin administration. Thus, patients with Bartter's syndrome exhibit a hyperadrenergic state consisten with resistance to endogenous, as well as exogenous, norepinephrine. Since the metabolic responses to intravenously administered norepinephrine were normal in the patient studied, norepinephrine resistance would appear to be limited to the vasculare system. Reversion of norepinephrine resistance during administration of an inhibitor of prostaglandin synthesis suggests that this hyperadrenergic state is not a primary pathogenetic abnormality in Bartter's syndrome.
在5例巴特综合征患者中，平均（±标准误）血浆去甲肾上腺素浓度从仰卧位时的324±75 pg/ml，分别在站立位2分钟、5分钟和10分钟后升至550±100 pg/ml、753±104 pg/ml和808±116 pg/ml，这些水平显著高于正常。血浆肾上腺素浓度与正常无异。1例患者在使用消炎痛治疗前对静脉注射去甲肾上腺素的升压（但非代谢）作用有抵抗，而在使用消炎痛期间恢复到正常的升压反应性。同样，该患者站立时内源性去甲肾上腺素的过度反应（10分钟血浆值为1110 pg/ml）在使用消炎痛期间恢复正常（10分钟值为462 pg/ml）。因此，巴特综合征患者表现出一种高肾上腺素能状态，对内源性和外源性去甲肾上腺素均有抵抗。由于在研究的患者中对静脉注射去甲肾上腺素的代谢反应正常，去甲肾上腺素抵抗似乎仅限于血管系统。在给予前列腺素合成抑制剂期间去甲肾上腺素抵抗的逆转表明，这种高肾上腺素能状态不是巴特综合征的原发性致病异常。
