Congenital Bile Acid Synthesis Defect 4 C1858328
A genetic condition impairing bile acid production, leading to liver disease and poor nutrient absorption. Managed with specialized bile acid replacement therapy.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- severe
- Chronic
- true
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy