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Acceptability study of a new paediatric formulation of Orphacol® (cholic acid) in paediatric patients treated for inborn errors in primary bile acid synthesis due to 3ß-Hydroxy-?5-C27- steroid oxidoreductase deficiency or ?4-3-Oxosteroid-5ß-reductase deficiency.

Not Applicable
Conditions
K76.8
Other specified diseases of liver
Registration Number
DRKS00013645
Lead Sponsor
aboratoires CTRS
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Complete
Sex
All
Target Recruitment
4
Inclusion Criteria

Males and females between 5 and 13 years old included.
Subject currently treated by Orphacol® commercial capsules or other primary bile acids for inborn errors in primary bile acid synthesis due to 3ß-Hydroxy-?5-C27-steroid oxidoreductase deficiency or ?4-3-Oxosteroid-5ß-reductase deficiency.

Exclusion Criteria

History of significant or ongoing psychiatric disorder (e.g. severe depression, schizophrenia, psychosis or manic depression).
Use of sedatives or antidepressants (including St. John’s Wort).
Sensory disorders affecting the mouth or local anaesthetics into the mouth within 48 hours before the study.
Previous surgery or radiotherapy for head and neck cancer.
Subject who has participated in another study and received any other investigational agent within 30 days prior to start of clinical dosing.
Subject and parents/legal representatives unable to provide written informed consent.
Failure to satisfy the investigator or eligibility to participate for any other reason.

Study & Design

Study Type
interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
To document patient acceptability of the Orphacol® dispersible tablet.
Secondary Outcome Measures
NameTimeMethod
To document potential difficulties encountered by the parents during preparation and administration of the product.
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