The syndrome of hypertension and hyperkalemia with normal GFR (Gordon's syndrome): is there increased proximal sodium reabsorption?
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.
Klemm
S A
SA
Endocrine-Hypertension Research Unit, Greenslopes Hospital, Brisbane, Australia.
Gordon
R D
RD
Tunny
T J
TJ
Thompson
R E
RE
D000042:Absorption; D000328:Adult; D000450:Aldosterone / Q000097:blood; D009320:Atrial Natriuretic Factor / Q000097:blood; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D006947:Hyperkalemia / Q000150:complications / Q000378:metabolism*; D006973:Hypertension / Q000150:complications / Q000378:metabolism*; D007687:Kidney Tubules, Proximal / Q000378:metabolism*; D008094:Lithium / Q000652:urine; D008297:Male; D008657:Metabolic Clearance Rate; D011188:Potassium / Q000097:blood / Q000652:urine; D012083:Renin / Q000097:blood; D012964:Sodium / Q000378:metabolism*; D013577:Syndrome
D000450:Aldosterone; D009320:Atrial Natriuretic Factor; D008094:Lithium; D012964:Sodium; D012083:Renin; D011188:Potassium