CHOLBAM (cholic acid)
DEFICIENCY OF BILE ACIDS DUE TO PEROXISOMAL DISORDER·+1
MANCHESTER PHAR
Indication
Deficiency of bile acids due to peroxisomal disorder
Deficiency of bile acids due to peroxisomal disorder is an inborn metabolic condition resulting from impaired hepatic peroxisomal beta-oxidation, which disrupts the side-chain shortening of cholesterol intermediates needed to produce mature primary bile acids like cholic acid. Read more about Deficiency of bile acids due to peroxisomal disorder
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Common Deficiency of bile acids due to peroxisomal disorder therapies.
CHOLBAM (cholic acid)
CHOLERETICS·DEFICIENCY OF BILE ACIDS DUE TO PEROXISOMAL DISORDER·+1
MIRUM PHARMACEU
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Coming soonDeficiency of bile acids due to peroxisomal disorder is an inborn metabolic condition resulting from impaired hepatic peroxisomal beta-oxidation, which disrupts the side-chain shortening of cholesterol intermediates needed to produce mature primary bile acids like cholic acid.
Commonly associated with peroxisome biogenesis defects such as Zellweger spectrum disorders or single peroxisomal enzyme deficiencies, it predominantly affects pediatric and adult populations presenting with hepatic and gastrointestinal impairment.
Pathophysiologically, the absence of functional peroxisomal enzymes leads to a marked depletion of normal C24 bile acids alongside the toxic accumulation of cholestanoic acid intermediates (such as di- and trihydroxycholestanoic acid).
Clinically, this manifests as cholestatic liver disease, steatorrhea, failure to thrive, and severe fat-soluble vitamin malabsorption.
- nih.gov
Deficiency of bile acids due to peroxisomal disorder is an inborn metabolic condition resulting from impaired hepatic peroxisomal beta-oxidation, which disrupts the side-chain shortening of cholesterol intermediates needed to produce mature primary bile acids like cholic acid Pathophysiologically, the absence of functional peroxisomal enzymes leads to a marked depletion of normal C24 bile acids alongside the toxic accumulation of cholestanoic acid intermediates (such as di- and trihydroxycholest...
- fda.gov
Deficiency of bile acids due to peroxisomal disorder is an inborn metabolic condition resulting from impaired hepatic peroxisomal beta-oxidation, which disrupts the side-chain shortening of cholesterol intermediates needed to produce mature primary bile acids like cholic acid
- mayocliniclabs.com
Commonly associated with peroxisome biogenesis defects such as Zellweger spectrum disorders or single peroxisomal enzyme deficiencies, it predominantly affects pediatric and adult populations presenting with hepatic and gastrointestinal impairment
- fiercebiotech.com
Commonly associated with peroxisome biogenesis defects such as Zellweger spectrum disorders or single peroxisomal enzyme deficiencies, it predominantly affects pediatric and adult populations presenting with hepatic and gastrointestinal impairment Clinically, this manifests as cholestatic liver disease, steatorrhea, failure to thrive, and severe fat-soluble vitamin malabsorption
- semanticscholar.org
Pathophysiologically, the absence of functional peroxisomal enzymes leads to a marked depletion of normal C24 bile acids alongside the toxic accumulation of cholestanoic acid intermediates (such as di- and trihydroxycholestanoic acid) Clinically, this manifests as cholestatic liver disease, steatorrhea, failure to thrive, and severe fat-soluble vitamin malabsorption
- nih.gov
Pathophysiologically, the absence of functional peroxisomal enzymes leads to a marked depletion of normal C24 bile acids alongside the toxic accumulation of cholestanoic acid intermediates (such as di- and trihydroxycholestanoic acid)
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