Klemm S A, Gordon R D, Tunny T J, Thompson R E
Endocrine-Hypertension Research Unit, Greenslopes Hospital, Brisbane, Australia.
Clin Invest Med. 1991 Dec;14(6):551-8.
The syndrome of hypertension and hyperkalemia, hyperchloremic acidosis with normal glomerular filtration rate (Gordon's syndrome) is characterised by volume expansion, suppressed renin and reduced mineralocorticoid-induced renal clearance of potassium. The clinical and biochemical defects are aggravated by high salt diet and corrected by low salt diet, leading to the hypothesis of excessive sodium reabsorption in the nephron proximal to where aldosterone acts. In this study, we used lithium clearance as a marker of proximal sodium reabsorption in three patients with Gordon's syndrome, in order to further localise the site in the nephron of defective sodium handling. Fractional excretion of lithium was decreased, and absolute and fractional proximal reabsorption of sodium was increased compared to normal controls. In addition, absolute distal reabsorption of sodium was decreased, consistent with decreased mineralocorticoid activity. Fractional excretion of potassium was markedly decreased and did not rise with increased distal delivery of sodium during saline infusion. However, after severe dietary sodium restriction had elevated plasma aldosterone (lowering plasma potassium levels to normal), fractional excretion of potassium was raised by saline infusion. Reduced lithium clearance in patients with Gordon's syndrome supports the hypothesis of increased proximal sodium reabsorption in this condition.
高血压与高钾血症、伴有正常肾小球滤过率的高氯性酸中毒综合征（戈登综合征）的特征为血容量扩张、肾素分泌受抑制以及盐皮质激素诱导的肾脏排钾减少。高盐饮食会加重临床和生化缺陷，而低盐饮食则可纠正这些缺陷，这导致了一种假说，即醛固酮作用部位近端的肾单位存在钠重吸收过多的情况。在本研究中，我们将锂清除率作为近端钠重吸收的标志物，对三名戈登综合征患者进行了研究，以便进一步确定肾单位中钠处理缺陷的部位。与正常对照组相比，锂的分数排泄降低，钠的绝对近端重吸收和分数近端重吸收增加。此外，钠的绝对远端重吸收减少，这与盐皮质激素活性降低一致。钾的分数排泄显著降低，在输注生理盐水期间，随着远端钠输送增加，钾的分数排泄并未升高。然而，在严格限制饮食中的钠使血浆醛固酮升高（将血浆钾水平降至正常）后，输注生理盐水可使钾的分数排泄增加。戈登综合征患者锂清除率降低支持了在这种情况下近端钠重吸收增加的假说。
