title: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles.
abstract: 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.
authors: Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, ICBS, UFRGS, Porto Alegre, RS, Brazil. aletheagatto@gmail.com
authors: Alethéa G
authors: AG
authors: Barschak
authors: Christiane
authors: C
authors: Marchesan
authors: Angela
authors: A
authors: Sitta
authors: Marion
authors: M
authors: Deon
authors: Roberto
authors: R
authors: Giugliani
authors: Moacir
authors: M
authors: Wajner
authors: Carmen Regla
authors: CR
authors: Vargas
mesh_terms: 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
chemical_list: 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