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High plasma leucine concentrations without neurological symptoms in a patient with classical maple syrup urine disease
Author(s) -
Skladal D.,
Grissenauer G.,
Konstantopoulou V.,
Felker S.,
Sperl W.
Publication year - 2000
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005600930139
Subject(s) - maple syrup urine disease , leucine , maple , urine , amino acid , chemistry , medicine , endocrinology , biochemistry , biology , botany
It has been found that the amino acid analyser used in this study systematically overestimated plasma leucine at high concentrations. The concentration reported as 2249 μmol/L had a true value of 1430 μmol/L and leucine values reported as >2000 μmol/L were approximately 1500 μmol/L.