1.
J Pediatr
; 92(5): 813-7, 1978 May.
Artigo
em Inglês
| MEDLINE
| ID: mdl-641635
RESUMO
The therapeutic efficacy of oral glycine was tested in a 3-year-old girl with isovaleric acidemia. An oral leucine load (25 mg/kg) caused a rise of the blood levels of isovaleric, lactic, and pyruvic acids as well as an increase of urinary excretion of the ketone bodies. These changes did not occur when oral glycine (250 mg/kg) was given with the leucine. Glycine supplementation favored the formation of isovalerylglycine, a nontoxic conjugate of isovaleric acid which is excreted rapidly. Excretion of isovalerylglycine rose threefold when leucine and glycine were administered simultaneously. Chronic glycine therapy was tolerated well and may have prevented one episode of ketoacidosis.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/terapia , Glicina/uso terapêutico , Valeratos/sangue , Acidose/terapia , Administração Oral , Pré-Escolar , Esquema de Medicação , Feminino , Glicina/administração & dosagem , Glicina/análogos & derivados , Glicina/urina , Humanos , Cetoácidos , Corpos Cetônicos/urina , Lactatos/sangue , Leucina/metabolismo , Piruvatos/sangue
2.
J Pediatr
; 79(1): 55-60, 1971 Jul.
Artigo
em Inglês
| MEDLINE
| ID: mdl-5091268