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THERAPEUTIC ATTEMPTS IN INFANTS WITH NONKETOTIC HYPERGLYCINAEMIA
Author(s) -
PUESCHEL S. M.,
CHA C.J. M.,
LANGON T.
Publication year - 1981
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/j.1365-2788.1981.tb00093.x
Subject(s) - strychnine , glycine , antagonist , hyperglycinemia , sodium , medicine , glycine receptor , endocrinology , pharmacology , anesthesia , receptor , chemistry , biochemistry , amino acid , organic chemistry
Identical male twins with non-ketotic hyperglycinaemia did not exhibit any development progress while on strychnine treatment. Since other therapeutic attempts with single agents previously had failed in most instances, we tried a combination of various treatment modalities. The results indicated that the administration of glycine receptor antagonist (strychnine) together with a low-protein diet (1.5 gm/kg/day), augmentation of 1-C units (N(5)-formlytetrahydrofolate), and removal of glycine from extracellular fluid (sodium salicylate and sodium benzoate) yielded some clinical improvement and positive biochemical changes in out patients.