Güllner H G
Klin Wochenschr. 1984 Oct 15;62(20):996-7. doi: 10.1007/BF01728431.
Patients with hypokalemia due to Bartter's syndrome show an increase in adrenergic nervous system function with significantly elevated plasma norepinephrine excretion. Prolonged exposure to neurotransmitters or hormones is known to lead to a down-regulation of target-cell responsiveness. We measured cyclic AMP generation by leukocytes in response to the beta-adrenergic agonist isoproterenol and to prostaglandin E1 (PGE1) in six patients with Bartter's syndrome. As compared to normal controls, the response of cyclic AMP production by leukocytes to stimulation by 1-isoproterenol or PGE1 was significantly decreased. These results indicate that in Bartter's syndrome and probably in other diseases involving hypokalemia isoproterenol- and PGE1-sensitive adenylate cyclase activities of leukocytes are reduced. Because the effect of PGE1 on adenylate cyclase is not mediated through the specific beta-adrenoceptor, it is possible that a defect in receptor-adenylate cyclase coupling or a more distal post-receptor defect is responsible for the reduction in cyclic AMP production.
巴特综合征所致低钾血症患者的肾上腺素能神经系统功能增强，血浆去甲肾上腺素排泄显著升高。已知长期暴露于神经递质或激素会导致靶细胞反应性下调。我们测定了6例巴特综合征患者白细胞对β-肾上腺素能激动剂异丙肾上腺素和前列腺素E1（PGE1）的环磷酸腺苷生成情况。与正常对照相比，白细胞对1-异丙肾上腺素或PGE1刺激的环磷酸腺苷产生反应显著降低。这些结果表明，在巴特综合征以及可能在其他涉及低钾血症的疾病中，白细胞对异丙肾上腺素和PGE1敏感的腺苷酸环化酶活性降低。由于PGE1对腺苷酸环化酶的作用不是通过特定的β-肾上腺素受体介导的，因此受体-腺苷酸环化酶偶联缺陷或更下游的受体后缺陷可能是环磷酸腺苷产生减少的原因。
