National Reyes Syndrome Foundation UK

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Inherited Metabolic Disorders Which Can Simulate Reye's Syndrome

Defects in Metabolism of Branched Chain Amino Acids

Disorders of Branched Chain Amino Acids
(valine, leucine, isoleucine & related organic acidaemias)

  • Maple Syrup Disease
       also known as:
       Maple Syrup Urine Disease
  • Multiple Carboxylase Deficiency
  • Isovaleric Acidaemia
  • Propionic Acidaemia
  • Methylmalonic Acidaemia
  • 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (3)
  • Mitochondrial Acetoacetyl-CoA Thiolase Deficiency
       also known as:
       Beta-Ketothiolase Deficiency
  • 3-Methylglutaconic Aciduria
    see note iii


Note iii

Methylglutaconic Aciduria (3) is listed on the CLIMB website individually under types 1, 2, 3 and 4.