E74.4 Disorders of pyruvate metabolism and gluconeogenesis

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

Applicable to

• Deficiency of phosphoenolpyruvate carboxykinase
• Deficiency of pyruvate carboxylase
• Deficiency of pyruvate dehydrogenase

Excludes1 (never code together)

• disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
• Leigh's syndrome (G31.82)

Applies from E74 Other disorders of carbohydrate metabolism

Excludes1 (never code together)

• diabetes mellitus (E08-E13)
• hypoglycemia NOS (E16.2)
• increased secretion of glucagon (E16.3)
• mucopolysaccharidosis (E76.0-E76.3)

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)

• 271.0 Glycogenosis (approximate)
• 271.8 Other specified disorders of carbohydrate transport and metabolism (approximate)

Code annotations containing back-references to E74.4

Back-references to E74.4

• Type 1 Excludes: E88.4 Mitochondrial metabolism disorders ("disorders of pyruvate metabolism (E74.4)")

Back-references to E74

• 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 carbohydrate metabolism (E73-E74)")

Diagnosis Index entries for E74.4

• Deficiency, deficient › phosphoenolpyruvate carboxykinase
• Deficiency, deficient › pyruvate › carboxylase
• Deficiency, deficient › pyruvate › dehydrogenase
• Disorder (of) › gluconeogenesis
• Disorder (of) › metabolism NOS › pyruvate
• Disorder (of) › pyruvate metabolism

Nearby codes

• E74.21 Galactosemia
• E74.29 Other disorders of galactose metabolism
• E74.3 Other disorders of intestinal carbohydrate absorption
• E74.31 Sucrase-isomaltase deficiency
• E74.39 Other disorders of intestinal carbohydrate absorption
• E74.4 Disorders of pyruvate metabolism and gluconeogenesis (this code)
• E74.8 Other specified disorders of carbohydrate metabolism
• E74.81 Disorders of glucose transport, not elsewhere classified
• E74.810 Glucose transporter protein type 1 deficiency
• E74.818 Other disorders of glucose transport
• E74.819 Disorders of glucose transport, unspecified