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. 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