ICD-10-CM E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

ICD-10-CM Diagnosis Code (2025)

Official Description for E71

Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Coding Notes & Guidelines for E71

Excludes1: Not Coded Here
  • peroxisomal disorders (E71.5)
  • Refsum's disease (G60.1)
  • Schilder's disease (G37.0)
Excludes1: Not Coded Here

glutaric aciduria (type 1) NOS (E72.3)

Excludes1: Not Coded Here

Muscle carnitine palmitoyltransferase deficiency (E71.314)

Excludes1: Not Coded Here

Schilder's disease (G37.0)

Excludes1: Not Coded Here

Refsum's disease (G60.1)

Excludes1: Not Coded Here

X-linked adrenoleukodystrophy (E71.42-)

Excludes1: Not Coded Here

chondrodysplasia punctata NOS (Q77.3)

Excludes2: Not Included Here

carnitine deficiency due to inborn error of metabolism (E71.42)

Inclusion Terms
  • Hyperleucine-isoleucinemia
  • Hypervalinemia
Inclusion Terms
  • LCAD deficiency
  • VLCAD deficiency
Inclusion Terms

MCAD deficiency

Inclusion Terms

SCAD deficiency

Inclusion Terms
  • Glutaric aciduria type II A
  • Glutaric aciduria type II B
  • Glutaric aciduria type II C
Inclusion Terms
  • Carnitine deficiency due to hemodialysis
  • Carnitine deficiency due to Valproic acid therapy
Inclusion Terms

Group 1 peroxisomal disorders

Inclusion Terms
  • Addison only phenotype adrenoleukodystrophy
  • Addison-Schilder adrenoleukodystrophy
Code Also

associated inborn error or metabolism

Child Codes (More Specific Codes for E71):

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 E71.19 - Other disorders of branched-chain amino-acid 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

About ICD-10-CM Code E71 (Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism)

ICD-10-CM code E71 is a billable or specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM E71 became effective on October 1, 2024.

This page provides detailed information about E71 - Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. It includes common terminology, coding guidelines such as "Includes," "Excludes1," and "Excludes2" notes, and information on required 7th characters. Utilizing this information correctly is essential for accurate medical coding and claims submission for conditions like Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism.