It is indicated for the treatment of inborn errors in primary bile-acid synthesis due to 3β-hydroxy-Δ5-C27-steroid oxidoreductase deficiency or Δ4-3-oxosteroid-5β-reductase deficiency in infants, children and adolescents aged one month to 18 years and adults.Cholic acid FGK (Kolbam) was approved for medical use in the European Union in November 2015. It is indicated for the treatment of inborn errors of primary bile acid synthesis, in infants from one month of age for continuous lifelong treatment through adulthood, encompassing the following single enzyme defects:
sterol 27-hydroxylase deficiency (presenting as cerebrotendinous xanthomatosis, CTX);
2- (or alpha-) methylacyl-CoA racemase (AMACR) deficiency;
cholesterol 7 alpha-hydroxylase (CYP7A1) deficiency.The most common side effects include peripheral neuropathy (nerve damage in the hands and feet), diarrhea, nausea (feeling sick), acid reflux (stomach acid flowing up into the mouth), esophagitis (inflammation of the food pipe), jaundice (yellowing of the skin and eyes), skin problems (lesions) and malaise (feeling unwell). Interactive pathway map
Click on genes, proteins and metabolites below to link to respective articles. References
External links
"Cholic acid". Drug Information Portal. U.S. National Library of Medicine.
The elimination half-life of oral emedastine in plasma is 3–4 hours, whereas that of topical emedastine is 10 hours. Approximately 44% of the oral dose is recovered in the urine over 24 hours with only 3.6% of the dose excreted as parent drug. Contraindications
Emedastine should not be used in patients who are hypersensitive to emedastine or any other excipients of the preparation. Benzalkonium chloride contained in the bottle of emedastine solution can discolor soft contact lenses, so people who wear contact lenses should be careful using it. Adverse events
The most common adverse effect was headache (11%). The other minor adverse effects encountered in less than 5% of patients were asthenia, burning or stinging sensation, unpleasant taste, blurred vision, eye dryness and tearing. == References ==