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Bile acid CoA ligase deficiency and defective amidation

ORPHA:276066· ICD-10 K76.8

Definition

Bile acid CoA ligase deficiency and defective amidation is an anomaly of bile acid synthesis characterized by fat malabsorption, neonatal cholestasis and growth failure.

Prevalence
Unknown
Inheritance
Unknown
Age of onset
Infancy, Neonatal