Medical Uses
Chenodeoxycholic Acid Leadiant is authorised in the European Union for inborn errors of primary bile acid synthesis due to sterol 27-hydroxylase deficiency, which presents as cerebrotendinous xanthomatosis (CTX), in infants, children, adolescents and adults. CTX is a rare inherited disorder in which cholestanol builds up in the brain, tendons and other tissues, causing diarrhoea, cataracts, tendon xanthomas and progressive neurological problems.
Chenodeoxycholic acid replaces the bile acid that patients with CTX cannot make. This switches off the abnormal over-production of bile acid precursors through feedback inhibition, lowering cholestanol levels and helping to stabilise or improve symptoms when started early. The usual adult dose is 750 mg per day divided into three doses.
