Posology : מינונים

4.2    Posology and method of administration

Treatment must be initiated and monitored by physicians experienced in the management of CTX or inborn errors of primary bile acid synthesis.

During the initiation of therapy and dose adjustment, serum cholestanol levels and/or urine bile alcohols should be monitored every 3 months till metabolic control and then annually. The lowest dose of chenodeoxycholic acid that effectively reduces the serum cholestanol and/or urine bile alcohols levels to within the normal range should be chosen. Liver function should also be monitored.
Concurrent elevation of liver enzymes above normal levels may indicate overdose. After the initiation period, cholestanol, urine bile alcohols and liver function should be determined annually, at a minimum, and the dose adjusted accordingly (see section 4.4). Additional or more frequent investigations may need to be undertaken to monitor therapy during periods of fast growth, concomitant disease and pregnancy (see section 4.6).

In case of persistent lack of therapeutic response to chenodeoxycholic acid monotherapy, other treatment options should be considered.

Posology
Adults
The starting dose in adults is 750 mg/day, in three divided doses provided that it is sufficient to normalise serum cholestanol and/or urine bile alcohols. The daily dose can be subsequently increased in 250 mg increments to a maximum of 1,000 mg/ day if the serum cholestanol and/or urine bile alcohols remain elevated.


Paediatric population (1 month -18 years)
The starting dose in children is 5 mg/kg/day in three divided doses. Where the dose calculated is not a multiple of 250 mg, the nearest dose below the maximum of 15 mg/kg/day should be selected, provided that is sufficient to normalise serum cholestanol and/or urine bile alcohols.

Neonates less than one month of age
The safety and efficacy in neonates less than one month of age have not been established. Limited safety data are available (see section 4.8).

Missed dose
If a dose is missed, the patient should take the next dose at the scheduled time. A double dose should not be taken to make up for the missed dose.

Special populations
Elderly patients (≥ 65 years)
Dose adjustment is not necessary.

Renal impairment
No data are available for patients with renal impairment. However, these patients should be carefully monitored and the dose titrated individually.

Hepatic impairment
No data are available for patients with hepatic impairment. However, these patients should be carefully monitored and the dose titrated individually.

Method of administration
Oral use.
Chenodeoxycholic acid Leadiant capsules can be taken with or without food. The hard capsules should be taken whole with sufficient water at approximately the same time each day. For infants and children who cannot swallow capsules, the capsules may be carefully opened and the content added to sodium bicarbonate solution 8.4%, see section 6.6.