ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C50 — Inborn errors of amino acid or other organic acid metabolism
5C50.D — Disorders of branched-chain amino acid metabolism

ICD-11 5C50.D — Disorders of branched-chain amino acid metabolism

The diagnosis has no description.

The diagnosis includes nothing.

It excludes 6 items.

  • Methylmalonic acidaemia (5C50.E0)
  • Propionic acidaemia (5C50.E0)
  • Isovaleric acidaemia (5C50.E0)
  • 3-methylglutaconic aciduria (5C50.E0)
  • Developmental delay due to 2-methylbutyryl-CoA dehydrogenase deficiency (5C50.E0)
  • 3-hydroxyisobutyric aciduria (5C50.E0)

Diagnosis with code 5C50.D contains 3 clarifying diagnoses:

  1. 5C50.D0 — Maple-syrup-urine disease
  2. 5C50.DY — Other specified disorders of branched-chain amino acid metabolism
  3. 5C50.DZ — Disorders of branched-chain amino acid metabolism, unspecified

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