We describe a simple method for measuring orotic acid and uracil concentrat
ion in urine by capillary zone electrophoresis in 20 mM Na-borate buffer, p
H 9.2. The method was applied for studying a patient with HHH (hyperornithi
nemia, hyperammonemia and homocitrullinuria) syndrome. A high value of urac
il excretion was found during periods of relatively low orotic acid excreti
on and normal ammonemia. The orotic acid level in urine was increased by in
creasing protein intake. (C) 1999 Elsevier Science B.V. All rights reserved
.