Active Ingredient: Cholic acid
Cholic acid is indicated for the treatment of inborn errors in primary bile acid synthesis due to Δ4-3-Oxosteroid-5β-reductase deficiency in infants, children and adolescents aged 1 month to 18 years and adults.
For this indication, competent medicine agencies globally authorize below treatments:
Oral
5 - 15 mg per kg of body weight
From 5 To 15 mg per kg of body weight once every day
The daily dose ranges from 5 to 15 mg/kg in infants, children, adolescents and adults. In all age groups, the minimum dose is 50 mg and the dose is adjusted in 50 mg steps. In adults, the daily dose should not exceed 500 mg.
The daily dose may be divided if it consists of more than 50 mg in order to mimic the continuous production of cholic acid in the body and to reduce the number of capsules that need to be taken per administration.
During the initiation of therapy and dose adjustment, serum and/or urine bile acid levels should be monitored intensively (at least every three months during the first year of treatment, every six months during the second year) using suitable analytical techniques. The concentrations of the abnormal bile acid metabolites synthesised in 3β-Hydroxy-Δ5-C27-steroid oxidoreductase deficiency (3β, 7α-dihydroxy- and 3β, 7α, 12α-trihydroxy-5-cholenoic acids) or in Δ4-3-Oxosteroid-5β-reductase deficiency (3-oxo-7α-hydroxy- and 3-oxo-7α, 12α-dihydroxy-4-cholenoic acids) should be determined. At each investigation, the need for dose adjustment should be considered. The lowest dose of cholic acid that effectively reduces the bile acid metabolites to as close to zero as possible should be chosen.
Patients that have previously been treated with other bile acids or other cholic acid preparations should be closely monitored in the same manner during the initiation of treatment with cholic acid. The dose should be adjusted accordingly, as described above.
Liver parameters should also be monitored, preferentially more frequently than serum and/or urine bile acid levels. Concurrent elevation of serum gamma glutamyltransferase (GGT), alanine aminotransferase (ALT) and/or serum bile acids above normal levels may indicate overdose. Transient elevations of transaminases at the initiation of cholic acid treatment have been observed and do not indicate the need for a dose reduction if GGT is not elevated and if serum bile acid levels are falling or in the normal range.
After the initiation period, serum and/or urine bile acids (using suitable analytical techniques) and liver parameters should be determined annually, at a minimum, and the dose adjusted accordingly. Additional or more frequent investigations should be undertaken to monitor therapy during periods of fast growth, concomitant disease and pregnancy
It must be taken with food at approximately the same time each day, in the morning and/or evening. Administration with food may increase cholic acid bioavailability and improve tolerability. Regular and fixed times of administration support the patient’s or caregiver’s compliance.
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