Specific code 2015 ICD-9-CM Diagnosis Code 270.3
Disturbances of branched-chain amino-acid metabolism
  • 2015
  • Billable Thru Sept 30/2015
  • Non-Billable On/After Oct 1/2015

  • ICD-9-CM 270.3 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 270.3 should only be used for claims with a date of service on or before September 30, 2015. For claims with a date of service on or after October 1, 2015, use an equivalent ICD-10-CM code (or codes).
Convert to ICD-10-CM: 270.3 converts approximately to:
  • 2015/16 ICD-10-CM E71.0 Maple-syrup-urine disease
  • 2015/16 ICD-10-CM E71.120 Methylmalonic acidemia
  • 2015/16 ICD-10-CM E71.19 Other disorders of branched-chain amino-acid metabolism
  • 2015/16 ICD-10-CM E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
Approximate Synonyms
  • 3-methylglutaconic aciduria
  • Aciduria, 3 methylgluaconic
  • Branched chain amino acid metabolism disorder
  • Cobalamin C disease
  • Disorder of branched-chain amino acid metabolism
  • Maple syrup urine disease
  • Methylmalonic acidemia
  • Methylmalonic acidemia, cobalamin C type
  • Propionic acidemia
Clinical Information
  • Autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids; metabolites accumulate in body fluids and render a "maple syrup" odor; divided into classic, intermediate, intermittent, and thiamine responsive subtypes; classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia; the intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting
Applies To
  • Disturbances of metabolism of leucine, isoleucine, and valine
  • Hypervalinemia
  • Intermittent branched-chain ketonuria
  • Leucine-induced hypoglycemia
  • Leucinosis
  • Maple syrup urine disease
ICD-9-CM Volume 2 Index entries containing back-references to 270.3:
  • Aciduria 791.9
    • methylmalonic 270.3
  • Branched-chain amino-acid disease 270.3
  • Degradation disorder, branched-chain amino-acid 270.3
  • Disease, diseased - see also Syndrome
    • maple syrup (urine) 270.3
  • Disorder - see also Disease
    • branched-chain amino-acid degradation 270.3
    • degradation, branched-chain amino acid 270.3
  • Disturbance - see also Disease
    • amino acid (metabolic) (see also Disorder, amino acid) 270.9
      • imidazole 270.5
      • maple syrup (urine) disease 270.3
      • transport 270.0
  • Hypervalinemia 270.3
  • Hypoglycemia (spontaneous) 251.2
    • leucine-induced 270.3
  • Ketonuria 791.6
    • branched-chain, intermittent 270.3
  • Leucinosis 270.3
  • Maple syrup (urine) disease or syndrome 270.3
  • Syndrome - see also Disease
    • maple syrup (urine) 270.3
    • Menkes' 759.89
      • glutamic acid 759.89
      • maple syrup (urine) disease 270.3
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 270.3 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2015 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.