Urinary excretion of 2-methyl-2,3-butanediol and 2,3-pentanediol in patients with disorders of propionate and methylmalonate metabolism.
Clin Chim Acta
; 165(2-3): 197-204, 1987 Jun 15.
Article
em En
| MEDLINE
| ID: mdl-3652445
ABSTRACT
Urine samples from patients with propionic acidemia and from a patient with methylmalonic acidemia contained unknown non-acidic metabolites by gas chromatography/mass spectrometry after ethyl acetate extraction. It could be demonstrated by mass spectrometric studies and by synthesis of reference compounds that the major metabolite was 2-methyl-2,3-butanediol, while smaller amounts of 2,3-pentanediol were also present. These diols were present in abnormal amounts in these patients during attacks of metabolic decompensation.
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Base de dados:
MEDLINE
Assunto principal:
Propionatos
/
Acidose
/
Butileno Glicóis
/
Glicóis
/
Malonatos
Limite:
Humans
Idioma:
En
Ano de publicação:
1987
Tipo de documento:
Article