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Diagnosis Code

E71.19 OTHER DISORDERS OF BRANCHED-CHAIN AMINO-ACID METABOLISM


Code Information

Diagnosis Code: E71.19

Short Description: Other disorders of branched-chain amino-acid metabolism

Long Description: Other disorders of branched-chain amino-acid metabolism

The code E71.19 is VALID for claim submission

Code Classification:

  • Endocrine, nutritional and metabolic diseases (E00–E89)
    • Metabolic disorders (E70-E88)
      • Disord of branched-chain amino-acid metab & fatty-acid metab (E71)
        • E71.19 Other disorders of branched-chain amino-acid metabolism

Code Version: 2022 ICD-10-CM


Synonyms

  • 2-methylbutyrylglycinuria
  • 3-Hydroxyisobutyric aciduria
  • Autism epilepsy syndrome due to branched chain ketoacid dehydrogenase kinase deficiency
  • Autistic disorder of childhood onset
  • Clinical manifestation of enzyme deficiency
  • Combined malonic and methylmalonic aciduria
  • Deficiency of acetyl-CoA acetyltransferase
  • Deficiency of hydroxymethylglutaryl-CoA lyase
  • Deficiency of isoleucine-tRNA ligase
  • Deficiency of leucine aminotransferase
  • Deficiency of leucine-tRNA ligase
  • Deficiency of valine-tRNA ligase
  • Developmental delay due to methylmalonate semialdehyde dehydrogenase deficiency
  • Disorder of isoleucine metabolism
  • Disorder of valine metabolism
  • Hydroxymethylglutaric aciduria
  • Hyperleucine-isoleucinemia
  • Hyperleucinemia
  • Hypervalinemia
  • Intermittent branched-chain ketonuria
  • Isobutyrylglycinuria
  • Isoleucinosis
  • Leucine-induced hypoglycemia
  • Methylcrotonyl-CoA carboxylase deficiency
  • Mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency - potassium stimulated
  • Valinosis

References to Index of Diseases and Injuries

The code E71.19 has the following ICD-10-CM references to the Index of Diseases and Injuries
  • Inclusion Terms:
    • Hyperleucine-isoleucinemia
    • Hypervalinemia

Crosswalk Information

ICD-10 Code ICD-9 Code ICD-9 Description
E71.19 Right Arrow 270.3 Bran-chain amin-acid dis

This ICD-10 to ICD-9 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-10 Code ICD-10 Description
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71.0 Maple-syrup-urine disease
E71.1 Other disorders of branched-chain amino-acid metabolism
E71.11 Branched-chain organic acidurias
E71.110 Isovaleric acidemia
E71.111 3-methylglutaconic aciduria
E71.118 Other branched-chain organic acidurias
E71.12 Disorders of propionate metabolism
E71.120 Methylmalonic acidemia
E71.121 Propionic acidemia
E71.128 Other disorders of propionate metabolism
E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
E71.3 Disorders of fatty-acid metabolism
E71.30 Disorder of fatty-acid metabolism, unspecified
E71.31 Disorders of fatty-acid oxidation
E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
E71.311 Medium chain acyl CoA dehydrogenase deficiency
E71.312 Short chain acyl CoA dehydrogenase deficiency
E71.313 Glutaric aciduria type II
E71.314 Muscle carnitine palmitoyltransferase deficiency
E71.318 Other disorders of fatty-acid oxidation
E71.32 Disorders of ketone metabolism
E71.39 Other disorders of fatty-acid metabolism
E71.4 Disorders of carnitine metabolism
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
E71.43 Iatrogenic carnitine deficiency
E71.44 Other secondary carnitine deficiency
E71.440 Ruvalcaba-Myhre-Smith syndrome
E71.448 Other secondary carnitine deficiency
E71.5 Peroxisomal disorders
E71.50 Peroxisomal disorder, unspecified
E71.51 Disorders of peroxisome biogenesis
E71.510 Zellweger syndrome
E71.511 Neonatal adrenoleukodystrophy
E71.518 Other disorders of peroxisome biogenesis
E71.52 X-linked adrenoleukodystrophy
E71.520 Childhood cerebral X-linked adrenoleukodystrophy
E71.521 Adolescent X-linked adrenoleukodystrophy
E71.522 Adrenomyeloneuropathy
E71.528 Other X-linked adrenoleukodystrophy
E71.529 X-linked adrenoleukodystrophy, unspecified type
E71.53 Other group 2 peroxisomal disorders
E71.54 Other peroxisomal disorders
E71.540 Rhizomelic chondrodysplasia punctata
E71.541 Zellweger-like syndrome
E71.542 Other group 3 peroxisomal disorders
E71.548 Other peroxisomal disorders

This page was last updated on: 10/1/2023

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