E71.313 Glutaric aciduria type II

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applicable to

• Glutaric aciduria type II A
• Glutaric aciduria type II B
• Glutaric aciduria type II C

Excludes1 (never code together)

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

Applies from E71.3 Disorders of fatty-acid metabolism

Excludes1 (never code together)

• peroxisomal disorders (E71.5)
• Refsum's disease (G60.1)
• Schilder's disease (G37.0)

Excludes2 (not included here)

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

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 277.85 Disorders of fatty acid oxidation (approximate)

Code annotations containing back-references to E71.313

Back-references to E71.313

• Type 1 Excludes: E72.3 Disorders of lysine and hydroxylysine metabolism ("glutaric aciduria type II (E71.313)")

Back-references to E71.3

• Type 1 Excludes: E72 Other disorders of amino-acid metabolism ("fatty-acid metabolism (E71.3)")

Back-references to E71

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of amino-acid metabolism (E70-E72)")
• Type 1 Excludes: R79.83 Abnormal findings of blood amino-acid level ("disorders of amino-acid metabolism (E70-E72)")

Diagnosis Index entries for E71.313

• Aciduria › glutaric (type I) › type II

Nearby codes

• 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 (this code)
• 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