E83.821 ENPP1 deficiency causing generalized arterial calcification of infancy

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

Code also

• , if applicable, associated conditions such as:
• heart failure (I50.-)
• other secondary hypertension (I15.8)

Applies from E83 Disorders of mineral metabolism

Excludes1 (never code together)

• dietary mineral deficiency (E58-E61)
• parathyroid disorders (E20-E21)
• vitamin D deficiency (E55.-)

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) 299-301 in MDC 05.

Code annotations containing back-references to E83.821

Back-references to E83.82

• Type 2 Excludes: E83.3 Disorders of phosphorus metabolism and phosphatases ("disorders of pyrophosphate metabolism (E83.82-)")
• Type 2 Excludes: I70.9 Other and unspecified atherosclerosis ("disorders of pyrophosphate metabolism (E83.82-)")
• Type 2 Excludes: Q28.8 Other specified congenital malformations of circulatory system ("disorders of pyrophosphate metabolism (E83.82-)")
• Type 2 Excludes: Q82.8 Other specified congenital malformations of skin ("disorders of pyrophosphate metabolism (E83.82-)")

Back-references to E83

• Type 1 Excludes: E61 Deficiency of other nutrient elements ("disorders of mineral metabolism (E83.-)")
• 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: R79.0 Abnormal level of blood mineral ("disorders of mineral metabolism (E83.-)")

Diagnosis Index entries for E83.821

• Calcification › arterial › generalized, of infancy › with › ENPP1 deficiency
• Deficiency, deficient › ENPP1 › causing › generalized arterial calcification of infancy

Nearby codes

• E83.59 Other disorders of calcium metabolism
• E83.8 Other disorders of mineral metabolism
• E83.81 Hungry bone syndrome
• E83.82 Disorders of pyrophosphate metabolism
• E83.820 Generalized arterial calcification of infancy with unspecified genetic causality
• E83.821 ENPP1 deficiency causing generalized arterial calcification of infancy (this code)
• E83.822 ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
• E83.823 ABCC6 deficiency causing generalized arterial calcification of infancy
• E83.824 ABCC6 deficiency causing pseudoxanthoma elasticum
• E83.825 CD73 deficiency causing arterial calcification
• E83.89 Other disorders of mineral metabolism