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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study

Fig. 1

A Leucine concentrations over time for adults and children (n = 102). Leucine normalisation is indicated by a dotted line at 381 µmol/L. B Isoleucine and valine concentrations over time for all episodes (n = 102). Recommended goal ranges for MSUD patients are leucine 76–205 μmol/L, isoleucine 40–90 μmol/L, and valine 200–425 μmol/L. In practice, leucine values between 76 and 380 μmol/L were considered acceptable [17]

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172