" /> Intermittent maple-syrup-urine disease - CISMeF





Preferred Label : Intermittent maple-syrup-urine disease;

ICD-11 definition : This is an intermittent autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia.;

ICD-11 synonym : Intermittent branched chain ketoaciduria;

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This is an intermittent autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia.

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27/07/2025


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