Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
In Metabolic disorders (E70-E88), chapter 4: Endocrine, Nutritional and Metabolic Diseases
38 billable codes under E71: 31 are a CC; none map to an HCC.A CC or MCC (major CC) is a secondary diagnosis that raises the MS-DRG payment.
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Instructional notes
Also applies, from Metabolic disorders (E70-E88)
These notes sit on E70-E88 and apply to every code under it, including E71.
Excludes1: not coded here, use a different code
Excludes2: not included here, code separately if applicable
- Ehlers-Danlos syndromes (Q79.6-)
Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Covers diabetes mellitus coding, including type, insulin and drug use, pump malfunction and secondary diabetes, plus obesity and obesity class codes.
- Diabetes mellitus (page 39)
- Obesity (page 42)
All codes under E71
E71 can't be used on a claim. Pick a billable code below; grey headings group them.
- E71.0Maple-syrup-urine disease
- E71.1Other disorders of branched-chain amino-acid metabolism
- E71.11Branched-chain organic acidurias
- E71.110Isovaleric acidemia
- E71.1113-methylglutaconic aciduria
- E71.118Other branched-chain organic acidurias
- E71.12Disorders of propionate metabolism
- E71.120Methylmalonic acidemia
- E71.121Propionic acidemia
- E71.128Other disorders of propionate metabolism
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.3Disorders of fatty-acid metabolism
- E71.30Disorder of fatty-acid metabolism, unspecified
- E71.31Disorders of fatty-acid oxidation
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
- E71.311Medium chain acyl CoA dehydrogenase deficiency
- E71.312Short chain acyl CoA dehydrogenase deficiency
- E71.313Glutaric aciduria type II
- E71.314Muscle carnitine palmitoyltransferase deficiency
- E71.318Other disorders of fatty-acid oxidation
- E71.32Disorders of ketone metabolism
- E71.39Other disorders of fatty-acid metabolism
- E71.4Disorders of carnitine metabolism
- E71.40Disorder of carnitine metabolism, unspecified
- E71.41Primary carnitine deficiency
- E71.42Carnitine deficiency due to inborn errors of metabolism
- E71.43Iatrogenic carnitine deficiency
- E71.44Other secondary carnitine deficiency
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.5Peroxisomal disorders
- E71.50Peroxisomal disorder, unspecified
- E71.51Disorders of peroxisome biogenesis
- E71.510Zellweger syndrome
- E71.511Neonatal adrenoleukodystrophy
- E71.518Other disorders of peroxisome biogenesis
- E71.52X-linked adrenoleukodystrophy
- E71.520Childhood cerebral X-linked adrenoleukodystrophy
- E71.521Adolescent X-linked adrenoleukodystrophy
- E71.522Adrenomyeloneuropathy
- E71.528Other X-linked adrenoleukodystrophy
- E71.529X-linked adrenoleukodystrophy, unspecified type
- E71.53Other group 2 peroxisomal disorders
- E71.54Other peroxisomal disorders
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
E71 MS-DRG
As the principal diagnosis, all 38 billable codes under E71 group to MS-DRG 642 (v44.0).
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
Metabolic Disorders
Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues, such as your liver, muscles, and body fat.
Read more about metabolic disorders on MedlinePlusCode history
Valid since FY2016, when ICD-10-CM took effect on October 1, 2015. No changes since.