We report on a severely hypotrophic male twin with persistent diarrhoea and metabolic acidosis during the first 4 weeks of life, who showed a fivefold normal glutamate concentration in plasma. Further evaluation excluded major defects in amino acid metabolism and after 5 months glutamate concentrations returned to normal. Neither the dizygotic twin sibling nor the parents revealed any clinical abnormalities or acid base or amino acid disturbances.
Conclusion: Transient glutamic acidaemia seems to be an extremely rare condition in newborn infants and appears to be without negative impact on the physical and neurological development during the first months of life.