Semmekrot B, Monnens L, Theelen B G, Rascher W, Gabreëls F, Willems J
Department of Pediatrics, University of Nijmegen, The Netherlands.
Pediatr Nephrol. 1987 Jul;1(3):473-8. doi: 10.1007/BF00849256.
A 14-year-old boy with the syndrome of hypertension and hyperkalaemia with normal glomerular filtration rate (Gordon's syndrome) is described. The patient's clinical symptoms consisted of periodic paralysis, slight metabolic acidosis of the proximal type and hypercalciuria. Prostaglandin excretion was normal. Infusion of atrial natriuretic peptide had no effect on electrolyte excretion or glomerular function although a normal increase in cyclic guanosine monophosphate was demonstrated in plasma and urine. This lack of sensitivity to atrial natriuretic peptide offers a new pathophysiological concept in this syndrome. Treatment with hydrochlorothiazide was successful in this case.
本文描述了一名14岁患有高血压、高钾血症且肾小球滤过率正常的男孩(戈登综合征)。患者的临床症状包括周期性麻痹、轻度近端型代谢性酸中毒和高钙尿症。前列腺素排泄正常。尽管血浆和尿液中循环鸟苷单磷酸正常增加,但输注心钠素对电解质排泄或肾小球功能没有影响。对心钠素缺乏敏感性为该综合征提供了一个新的病理生理学概念。在该病例中,使用氢氯噻嗪治疗成功。