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d ‐GLYCERIC‐ACIDAEMIA AND NON‐KETOTIC HYPERGLYCINEMIA
Author(s) -
BRANDT N. J.,
RASMUSSEN K.,
BRANDT S.,
KØLVRAA S.,
SCHØNHEYDER F.
Publication year - 1976
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1976.tb04401.x
Subject(s) - hyperglycinemia , glyceric acid , medicine , urine , excretion , glycine , endocrinology , biochemistry , amino acid , chemistry , catalysis
. The clinical and laboratory findings in a 2½‐year‐old boy with non‐ketotic hyperglycinaemia are reported. Except for a somewhat impaired liver function there was a picture similar to the cases previously reported in the literature. The patient deviated from the classical description, however, in the excretion of large amounts of d ‐glyceric acid in the urine. The same compound was also repeatedly found in the serum. It is suggested that the large amounts of glycine found in various body fluids are secondary to a hitherto undescribed enzymatic defect in the degradation of d ‐glyceric acid.