E78.79
BillableOther disorders of bile acid and cholesterol metabolism
HCC Category Mapping
RxHCCHCC 47 — Disorders of Fatty-Acid and Lipid Metabolism
0.000What This Code Means
Other rare disorders affecting how the body processes bile acids and cholesterol that are not classified elsewhere.
Coding Tips
Clinical Significance
Other disorders of bile acid and cholesterol metabolism captures specific bile acid synthesis defects such as cerebrotendinous xanthomatosis (CTX), 3-beta-hydroxy-delta-5-C27-steroid oxidoreductase deficiency, and other inborn errors of bile acid synthesis. These conditions can be treatable with chenodeoxycholic acid replacement, making accurate diagnosis critically important.
Documentation Requirements
- ✓Specific bile acid or cholesterol metabolism disorder documented (e.g., cerebrotendinous xanthomatosis, specific bile acid synthesis defect)
- ✓Confirmatory diagnostic testing: serum cholestanol levels (for CTX), urine bile acid profile, genetic testing
- ✓Clinical manifestations specific to the identified disorder
- ✓Treatment plan: chenodeoxycholic acid or cholic acid replacement therapy
- ✓Provider statement confirming this is a metabolic disorder of bile acid/cholesterol pathway
Commonly Confused Codes
E78.70 — Disorder of bile acid and cholesterol metabolism, unspecified: use only when the specific disorder is unknownE75.5 — Other lipid storage disorders: for intracellular lipid accumulation, not bile acid pathway defectsE78.2 — Mixed hyperlipidemia: circulating lipid elevation, not bile acid synthesis defectG60.9 — Hereditary and idiopathic neuropathy, unspecified: CTX may present with neuropathy, but code the metabolic cause
Code Hierarchy
└E78Disorders of lipoprotein metabolism and other lipidemias└E78.7Disorders of bile acid and cholesterol metabolism└E78.79Other disorders of bile acid and cholesterol metabolism
└E78.79Other disorders of bile acid and cholesterol metabolism