Maple syrup urine disease in treated patients: Biochemical and oxidative stress profiles

被引：23

作者：

Barschak, Alethea G. ^{[1
,2
]}

Marchesan, Christiane ^{[3
]}

Sitta, Angela ^{[1
,2
]}

Deon, Marion ^{[1
,2
]}

Giugliani, Roberto ^{[1
]}

Wajner, Moacir ^{[1
,2
]}

Vargas, Carmen Regla ^{[1
,2
,3
]}

机构：

[1] HCPA, Serv Genet Med, BR-90035903 Porto Alegre, RS, Brazil

[2] Univ Fed Rio Grande do Sul, ICBS, Programa Posgrad Ciencias Biol Bioquim, Porto Alegre, RS, Brazil

[3] Univ Fed Rio Grande do Sul, PPGCF, Fac Farm, Dept Anal, Porto Alegre, RS, Brazil

来源：

CLINICAL BIOCHEMISTRY | 2008年 / 41卷 / 4-5期

关键词：

maple syrup urine disease; biochemical profile; lipid peroxidation; antioxidant reactivity;

D O I：

10.1016/j.clinbiochem.2007.11.015

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Objective: The objective of this study was to evaluate and correlate the biochemical and oxidative stress profiles in MSUD patients during the dietary treatment. Design and 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. (C) 2007 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

引用

页码：317 / 324

页数：8

共 28 条

[1] Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease [J].

Barschak, Alethea G. ;

Sitta, Angela ;

Deon, Marion ;

de Oliveira, Marcella H. ;

Haeser, Alexsandro ;

Dutra-Filho, Carlos S. ;

Wajner, Moacir ;

Vargas, Carmen R. .

METABOLIC BRAIN DISEASE, 2006, 21 (04) :279-286

[2] The free radical theory of aging matures [J].

Beckman, KB ;

Ames, BN .

PHYSIOLOGICAL REVIEWS, 1998, 78 (02) :547-581

[3] Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease [J].

Bridi, R ;

Araldi, J ;

Sgarbi, MB ;

Testa, CG ;

Durigon, K ;

Wajner, M ;

Dutra-Filho, CS .

INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE, 2003, 21 (06) :327-332

[4]

Buege J A, 1978, Methods Enzymol, V52, P302

[5]

BURTIS CA, 1998, TIETZ FUNDAMENTALS Q

[6]

Chuang DT., 2001, The metabolic and molecular bases of inherited disease, P1971

[7] Inborn errors of metabolism:: cause of oxidative stress? [J].

Colomé, C ;

Sierra, C ;

Vilaseca, A .

MEDICINA CLINICA, 2000, 115 (03) :111-117

[8] The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy [J].

Deon, Marion ;

Wajner, Moacir ;

Sirtori, Lisana R. ;

Fitarelli, Douglas ;

Coelho, Daniella M. ;

Sitta, Angela ;

Barschak, Alethea G. ;

Ferreira, Gustavo C. ;

Haeser, Alexsandro ;

Giugliani, Roberto ;

Vargas, Carmen R. .

JOURNAL OF THE NEUROLOGICAL SCIENCES, 2006, 247 (02) :157-164

[9]

ESTERBAUER H, 1990, METHOD ENZYMOL, V186, P407

[10] Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats [J].

Fontella, FU ;

Pulrolnik, V ;

Gassen, E ;

Wannmacher, CMD ;

Klein, AB ;

Wajner, M ;

Dutra, CS .

NEUROREPORT, 2000, 11 (03) :541-544

← 1 2 3 →