E72.3 Disorders of lysine and hydroxylysine metabolism

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

Applicable to

• Glutaric aciduria NOS
• Glutaric aciduria (type I)
• Hydroxylysinemia
• Hyperlysinemia

Excludes1 (never code together)

• glutaric aciduria type II (E71.313)
• Refsum's disease (G60.1)
• Zellweger syndrome (E71.510)

Applies from E72 Other disorders of amino-acid metabolism

Excludes1 (never code together)

• disorders of:
• aromatic amino-acid metabolism (E70.-)
• branched-chain amino-acid metabolism (E71.0-E71.2)
• fatty-acid metabolism (E71.3)
• purine and pyrimidine metabolism (E79.-)
• gout (M1A.-, M10.-)

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)

• 270.7 Other disturbances of straight-chain amino-acid metabolism (approximate)

Code annotations containing back-references to E72.3

Back-references to E72.3

• Type 1 Excludes: E71.313 Glutaric aciduria type II ("glutaric aciduria (type 1) NOS (E72.3)")

Back-references to E72

• 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 E72.3

• Acidemia › pipecolic
• Aciduria › glutaric (type I)
• Disorder (of) › lysine and hydroxylysine metabolism
• Disorder (of) › metabolism NOS › amino-acid › hydroxylysine
• Disorder (of) › metabolism NOS › amino-acid › lysine
• Disturbance (s) › metabolism › hydroxylysine
• Disturbance (s) › metabolism › lysine
• Glutaric aciduria
• Hydroxylysinemia
• Hyperaminoaciduria › lysine
• Hyperlysinemia
• Intolerance › lysine
• Lysine and hydroxylysine metabolism disorder
• Saccharopinuria

Nearby codes

• E72.20 Disorder of urea cycle metabolism, unspecified
• E72.21 Argininemia
• E72.22 Arginosuccinic aciduria
• E72.23 Citrullinemia
• E72.29 Other disorders of urea cycle metabolism
• E72.3 Disorders of lysine and hydroxylysine metabolism (this code)
• E72.4 Disorders of ornithine metabolism
• E72.5 Disorders of glycine metabolism
• E72.50 Disorder of glycine metabolism, unspecified
• E72.51 Non-ketotic hyperglycinemia
• E72.52 Trimethylaminuria