E74.4
BillableDisorders of pyruvate metabolism and gluconeogenesis
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A metabolic disorder affecting how the body produces energy from pyruvate or creates new glucose, leading to lactic acidosis, neurological problems, or low blood sugar.
Coding Tips
- •Specify the type of pyruvate metabolism disorder if documented (pyruvate dehydrogenase deficiency, etc.)
- •Note any complications such as lactic acidosis or neurological manifestations for additional coding
Clinical Significance
Disorders of pyruvate metabolism and gluconeogenesis include pyruvate dehydrogenase deficiency, pyruvate carboxylase deficiency, and phosphoenolpyruvate carboxykinase deficiency. These conditions disrupt central energy metabolism, causing severe lactic acidosis, hypoglycemia, and progressive neurological damage. They are among the most serious metabolic disorders, often presenting in the neonatal period with metabolic crisis.
Documentation Requirements
- ✓Specific pyruvate metabolism or gluconeogenesis enzyme deficiency identified
- ✓Laboratory confirmation including lactate levels, pyruvate levels, and lactate-to-pyruvate ratio
- ✓Genetic testing or enzyme assay results
- ✓Documentation of neurological findings: developmental delay, seizures, or encephalopathy
- ✓Current treatment plan including dietary management (ketogenic diet for PDH deficiency) and cofactor supplementation
Excludes 1 — Do NOT code together
Commonly Confused Codes
E87.2 — Acidosis: lactic acidosis is a manifestation of pyruvate disorders, not the primary diagnosisE74.4 specifically captures pyruvate metabolism defects, while E88.89 covers other metabolic disordersE74.00-E74.09 — Glycogen storage diseases: separate category of carbohydrate metabolism disordersG93.49 — Other encephalopathy: neurological damage is a complication, not the underlying metabolic defectE16.2 — Hypoglycemia, unspecified: hypoglycemia from gluconeogenesis defects should use E74.4 as primary
Code Hierarchy
└E74Other disorders of carbohydrate metabolism└E74.4Disorders of pyruvate metabolism and gluconeogenesis
└E74.4Disorders of pyruvate metabolism and gluconeogenesis