Therapeutic attempts in infants with nonketotic hyperglycinaemia.
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.
D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage; D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy*; D001565:Benzoates / Q000008:administration & dosage; D019817:Benzoic Acid; D002998:Clonazepam / Q000008:administration & dosage; D004044:Dietary Proteins / Q000008:administration & dosage; D004200:Diseases in Twins; D005998:Glycine / Q000097:blood*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D002955:Leucovorin / Q000008:administration & dosage; D008297:Male; D012980:Sodium Salicylate / Q000008:administration & dosage; D013331:Strychnine / Q000008:administration & dosage
D001565:Benzoates; D004044:Dietary Proteins; D002998:Clonazepam; D019817:Benzoic Acid; D000324:Adrenocorticotropic Hormone; D013331:Strychnine; D002955:Leucovorin; D005998:Glycine; D012980:Sodium Salicylate