Fasting plasma concentrations of branched-chain amino acids (BCAA) valine, leucine, and isoleucine were measured in 20 young patients (aged 18±2 years) with end-stage renal disease just before initiation of dialysis and compared with 7 healthy controls (aged 19±1 years). Plasma valine, leucine, and isoleucine were all lower than control values (PP3) treatment to correct the acidosis. To control for the effect of additional sodium, they were also studied after 2 weeks of an equivalent amount of oral sodium chloride (NaCl). Oral NaHCO3 treatment led to significant increases in venous pH and serum bicarbonate concentrations, but no significant change in total and ionized calcium, phosphate, sodium, potassium, creatinine, blood urea nitrogen, and intact parathyroid hormone concentrations. Oral NaCl did not change any of the biochemical parameters. Fasting plasma concentrations of BCAA were measured. Before treatment of acidosis, uremic patients had low plasma concentrations of valine, leucine, and isoleucine compared with controls. Following 2 weeks of NaHCO3 treatment, there were significant increases in the plasma concentrations of valine and leucine (P3 and NaCl. Thus treatment of metabolic acidosis ameliorated abnormalities in plasma concentrations of valine and leucine in patients with uremia on hemodialysis.