Chenodiol (3 brand names, 1 dosage form, 1 route)
Available dosage forms: TABLET.
Routes of administration: ORAL.
Brand Names
- CHENIX
- CHENODIOL
- CTEXLI
Indications and Usage
CTEXLI is indicated for the treatment of cerebrotendinous xanthomatosis (CTX) in adults.
Contraindications
None.
Adverse Reactions
The following clinically significant adverse reaction is described elsewhere in the labeling: •Hepatotoxicity [see Warnings and Precautions (5.1)]
Mechanism of Action
Endogenous chenodiol (chenodeoxycholic acid) is a primary bile acid, synthesized from cholesterol in the liver. In CTX, the major bile acid synthesis pathways are disrupted due to partial or total deficiency in sterol 27-hydroxylase encoded by the CYP27A1 gene. CTEXLI may act to replace deficient levels of the endogenous bile acid chenodeoxycholic acid in patients with CTX. Increased chenodiol levels in the enterohepatic bile acid pool restore the activation of farnesoid X receptor (FXR) and downregulate CYP7A1 leading to suppression and reduction of atypical bile acids and bile alcohols including cholestanol and 23S-pentol.
Overdosage
Cases of intentional overdose with chenodiol have been reported: one patient consumed 3 g to 4.5 g of chenodiol and another patient consumed 30 g of chenodiol. Clinical manifestations experienced by these patients included nausea, dizziness, and diarrhea. In the event of an overdose, discontinue CTEXLI, monitor the patient, and institute general supportive measures if needed.