CHOLBAM (cholic acid)
DEFICIENCY OF BILE ACIDS DUE TO PEROXISOMAL DISORDER·+1
MANCHESTER PHAR
Indication
Deficiency of bile acids in bile acid synthesis disorder
A deficiency of bile acids in bile acid synthesis disorders refers to an inborn error of metabolism where inherited single-enzyme defects disrupt the conversion of cholesterol into primary bile acids. Read more about Deficiency of bile acids in bile acid synthesis disorder
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CHOLBAM (cholic acid)
CHOLERETICS·DEFICIENCY OF BILE ACIDS DUE TO PEROXISOMAL DISORDER·+1
MIRUM PHARMACEU
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Coming soonA deficiency of bile acids in bile acid synthesis disorders refers to an inborn error of metabolism where inherited single-enzyme defects disrupt the conversion of cholesterol into primary bile acids.
Primarily affecting the hepatic and gastrointestinal systems in neonates, children, and occasionally adults, this condition impairs bile flow and disrupts the absorption of dietary lipids and fat-soluble vitamins.
Pathophysiologically, the resulting lack of essential primary bile acids triggers compensatory upregulation of upstream enzymes, leading to the accumulation of hepatotoxic intermediate metabolites and atypical bile acids.
Clinically, affected patients present with neonatal or late-onset cholestatic jaundice, failure to thrive, hepatomegaly, fat malabsorption, and potentially progressive liver failure or neurological complications.
- nih.gov
A deficiency of bile acids in bile acid synthesis disorders refers to an inborn error of metabolism where inherited single-enzyme defects disrupt the conversion of cholesterol into primary bile acids Primarily affecting the hepatic and gastrointestinal systems in neonates, children, and occasionally adults, this condition impairs bile flow and disrupts the absorption of dietary lipids and fat-soluble vitamins Pathophysiologically, the resulting lack of essential primary bile acids triggers compe...
- cincinnatichildrens.org
A deficiency of bile acids in bile acid synthesis disorders refers to an inborn error of metabolism where inherited single-enzyme defects disrupt the conversion of cholesterol into primary bile acids Primarily affecting the hepatic and gastrointestinal systems in neonates, children, and occasionally adults, this condition impairs bile flow and disrupts the absorption of dietary lipids and fat-soluble vitamins Pathophysiologically, the resulting lack of essential primary bile acids triggers compe...
- nih.gov
Clinically, affected patients present with neonatal or late-onset cholestatic jaundice, failure to thrive, hepatomegaly, fat malabsorption, and potentially progressive liver failure or neurological complications
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