Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles.
OBJECTIVE
The objective of this study was to evaluate and correlate the biochemical and oxidative stress profiles in MSUD patients during the dietary treatment.


METHODS
Plasma samples from treated MSUD patients were used to evaluate the biochemical profile and oxidative stress parameters.


RESULTS
It was observed that glucose, total cholesterol, albumin and creatinine are reduced and that aspartate aminotransferase and lactate dehydrogenase activities are increased in plasma from MSUD patients under treatment. Besides, it was verified an increase of thiobarbituric acid-reactive species (TBARS) and a decrease of total antioxidant reactivity (TAR).


CONCLUSIONS
Our results suggest that oxidative stress occurs in treated MSUD patients and that dietary treatment and clinical conditions associated to the disease can lead to biochemical alterations in these patients.
Barschak
Alethéa G
AG
Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, ICBS, UFRGS, Porto Alegre, RS, Brazil. aletheagatto@gmail.com
Marchesan
Christiane
C
Sitta
Angela
A
Deon
Marion
M
Giugliani
Roberto
R
Wajner
Moacir
M
Vargas
Carmen Regla
CR
D000975:Antioxidants / Q000378:metabolism; D002784:Cholesterol / Q000097:blood; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D003401:Creatine / Q000097:blood; D005947:Glucose / Q000378:metabolism; D006801:Humans; D006836:Hydro-Lyases / Q000097:blood; D007223:Infant; D007231:Infant, Newborn; D008375:Maple Syrup Urine Disease / Q000097:blood*; D018384:Oxidative Stress / Q000502:physiology*; D017392:Thiobarbituric Acid Reactive Substances / Q000378:metabolism; D000637:Transaminases / Q000097:blood; D014280:Triglycerides / Q000097:blood; D014508:Urea / Q000097:blood; D014527:Uric Acid / Q000097:blood
D000975:Antioxidants; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D017392:Thiobarbituric Acid Reactive Substances; D014280:Triglycerides; D014527:Uric Acid; D014508:Urea; D002784:Cholesterol; D000637:Transaminases; D006836:Hydro-Lyases; C045581:lactate dehydratase; D005947:Glucose; D003401:Creatine