Abstract
Abstract
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
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Armstrong MD, Stave U (1973) A study of plasma free amino acid levels. I. Study of factors affecting validity of amino acid analysis. Metabolism 22:549–560
Brodehl J, Gellissen K (1968) Endogenous renal transport of frec amino acids in infancy and childhood. Pediatrics 42:395–404
Brown JH, Fabre LF, Farell GL, Adams ED (1972) Hyperlysinuria with hyperammonemia. A new metabolic disorder. Am J Dis Child 124:127–132
Condon JR, Asatoor AM (1971) Amino acid metabolism in uraemic patients. Clin Chim Acta 32:333–337
Dickinson IC, Rosenblum H, Hamilton PB, (1965) Ion exchange chromatography of the free amino acids in the plasma of the newborn infant. Pediatrics 36:2–13
Gutman AB, Yü T-F (1973) Hyperglutamatemia in primary gout. Am J Med 54:713–724
Hagenfeldt L, Larsson A, Andersson R (1978) The gamma-glutamyl cycle and amino acid transport. Studies of free amino acids, gamma-glutamylcysteine and glutathion in erythrocytes from patients with 5-oxoprolinuria. N Engl J Med 299:587–590
Levy HL, Barkin E (1971) Comparison of amino acid concentrations between plasma and erythrocytes. Studies in normal human subjects and those with metabolic disorders. J Lab Clin Med 78:517–523
Melacon SB, Dallaire L, Lemieux B, Robitaille P, Potier M (1977) Dicarboxylic aminoaciduria: An inborn error of amino acid conversion. J Pediatr 91:422–427
Nicolopoulos D, Perakis A, Papdakis M, Alexiou D, Aravantinos D (1976) Estimation of gestational age in the neonate. Am J Dis Child 130:477–480
Pagliara AS, Goodman AD (1969) Elevation of plasma glutamate in gout. Its possible role in pathogenesis of hyperuricemia. N Engl J Med 281:767–770
Pohlandt F (1978) Plasma amino acids of newborn infants breastfed ad libitum. J Pediatr 92:614–616
Pohlandt F (1978) Plasma amino acid concentration in umbilical cord vein and artery of newborn infants after elective cesarean section or spontaneous delivery. J Pediatr 92:617–623
Pohlandt F (1994) Prevention of postnatal bone demineralization in very low-birth-weight infants by individually monitored supplementation with calcium and phosphorus. Pediatr Res 35:125–129
Rassin DK, Gaull GE, Heinonen K, Räihä NCR (1977) Milk protein quantity and quality in low-birth-weight infants. II. Effects on selected aliphatic amino acids in plasma and urine. Pediatrics 59:407–422
Teijema HL, Brubakk A-M, van Gelderen HH, Ruys JH (1977) Glutamic acidemia. Hum Hered 27:217–218
Wadlington W, Kilroy A, Ando T, Sweetman L, Nyhan WL (1975) Hyperglycinemia and propionyl CoA carboxylase deficiency and episodic severe illness without consistent ketosis. J Pediatr 86:707–712
Wilson H, Cannan RK (1939) The glutamic acid — pyrrolidone carboxylic acid system. J Biol Chem 119:309–331
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Franz, A.R., Pohlandt, F. Transient glutamic acidaemia. Eur J Pediatr 155, 308–310 (1996). https://doi.org/10.1007/BF02002718
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02002718