Maple Syrup Urine Disease (MSUD)

Definition

MSUD is a metabolic disease characterized by branched-chain amino acid metabolism disorder, maple syrup odor in the urine, and neurological impairment.

Symptoms

  • Maple syrup odor in urine
  • Neurological disorder
  • Vomiting
  • Lethargy

Reasons

BCKDHA, BCKDHB, DBT gene mutations.

Diagnosis

Plasma amino acids, genetic testing.

Treatment

Dietary restriction, thiamine.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of metabolic crisis, emergency services should be consulted.

This page is for general information purposes only.