Guanidinosuccinic Acid Excretion in Argininosuccinic Aciduria

Abstract

Guanidinosuccinic acid (GSA) is a normally occurring metabolite in serum and urine of human beings. An intact pathway of urea formation seems to be necessary for its formation because from different inborn defects of urea synthesis the excretion of only minute amounts of GSA is reported (Stein et al. 1969). States of nitrogen retention, especially renal failure, show increased concentrations of GSA in serum and urine (Stein et al. 1969). We want to present our data on the urinary GSA excretion in a patient with argininosuccinic aciduria during the first two weeks after the introduction of ketoanalogues of essential amino acids into the dietary regime.