Catalog

Congenital bile acid synthesis defect type 2

No MMS code

Link to WHO Definition

Congenital bile acid synthesis defect type 2 – http://id.who.int/icd/entity/383380992

Congenital bile acid synthesis defect type 2 (BAS defect type 2) is an anomaly of bile acid synthesis characterised by severe and rapidly progressive cholestatic liver disease, and malabsorption of fat and fat-soluble vitamins.