We report on a severely hypotrophic male twin with persistent diarrhoe
a and metabolic acidosis during the first 4 weeks of life, who showed
a fivefold normal glutamate concentration in plasma. Further evaluatio
n excluded major defects in amino acid metabolism and after 5 months g
lutamate concentrations returned to normal. Neither the dizygotic twin
sibling nor the parents revealed any clinical abnormalities or acid b
ase or amino acid disturbances. Conclusion Transient glutamic acidaemi
a seems to be an extremely rare condition in newborn infants and appea
rs to be without negative impact on the physical and neurological deve
lopment during the first months of life.