270.3 BRAN-CHAIN AMIN-ACID DIS


Code Information

Diagnosis Code: 270.3

Short Description: Bran-chain amin-acid dis

Long Description: Disturbances of branched-chain amino-acid metabolism

Code Classification:

  • Endocrine, nutritional and metabolic diseases, and immunity disorders (240–279)
    • Other metabolic disorders and immunity disorders (270-279)
      • 270 Disorders of amino-acid transport and metabolism
        • 270.3 Bran-chain amin-acid dis

Code Version: 2015 ICD-9-CM


References to Index of Diseases and Injuries

The code 270.3 has the following ICD-9-CM references to the Index of Diseases and Injuries
  • Aciduria 791.9
    • methylmalonic 270.3
      • with glycinemia 270.7
  • 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
    • amino acid (metabolic) (see also Disturbance, metabolism, amino acid) 270.9
      • maple syrup (urine) disease 270.3
    • 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
      • maple syrup (urine) disease 270.3
    • metabolism (acquired) (congenital) (see also Disorder, metabolism) 277.9
      • amino acid (see also Disorder, amino acid) 270.9
        • branched-chain 270.3
      • isoleucine 270.3
      • leucine 270.3
      • valine 270.3
  • 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
      • maple syrup (urine) disease 270.3

Crosswalk Information

The code 270.3 converts into the following ICD-10 code(s):
ICD-9 Code ICD-10 Code ICD-10 Description
270.3 Right Arrow E71.0 Maple-syrup-urine disease
E71.120 Methylmalonic acidemia
E71.19 Other disorders of branched-chain amino-acid metabolism
E71.2 Disorder of branched-chain amino-acid metabolism, unsp
This ICD-9 to ICD-10 data is based on the 2018 General Equivalency Mapping (GEM) files published by the Centers for Medicare & Medicaid Services (CMS) for informational purposes only. The data is not an ICD-10 conversion tool and doesn’t guarantee clinical accuracy.

Similar Codes

ICD-9 Code ICD-9 Description
270.0 Disturbances of amino-acid transport
270.1 Phenylketonuria [PKU]
270.2 Other disturbances of aromatic amino-acid metabolism
270.4 Disturbances of sulphur-bearing amino-acid metabolism
270.5 Disturbances of histidine metabolism
270.6 Disorders of urea cycle metabolism
270.7 Other disturbances of straight-chain amino-acid metabolism
270.8 Other specified disorders of amino-acid metabolism
270.9 Unspecified disorder of amino-acid metabolism


This page was last updated on: 10/1/2014