E70.0 Classical phenylketonuria

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

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 42 — Acromegaly 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)

• 270.1 Phenylketonuria [PKU] (approximate)

Code annotations containing back-references to E70.0

Back-references to E70

• Type 1 Excludes: E72 Other disorders of amino-acid metabolism ("aromatic amino-acid metabolism (E70.-)")
• 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 E70.0

• Disorder (of) › metabolism NOS › amino-acid › aromatic › hyperphenylalaninemia › classical phenylketonuria
• Følling's disease
• Oligophrenia › phenylpyruvic
• Phenylketonuria › classical

Nearby codes

• E70 Disorders of aromatic amino-acid metabolism
• E70.0 Classical phenylketonuria (this code)
• E70.1 Other hyperphenylalaninemias
• E70.2 Disorders of tyrosine metabolism
• E70.20 Disorder of tyrosine metabolism, unspecified
• E70.21 Tyrosinemia
• E70.29 Other disorders of tyrosine metabolism