Clinical trial • Phase III • Endocrinology | Rare Disease

Biotin for Methylcrotonylglycinuria | Biotinidase deficiency

Phase III trial of Biotin for Methylcrotonylglycinuria | Biotinidase deficiency.

Overview

Trial Therapeutic Area
Endocrinology | Rare Disease
Trial Disease
Methylcrotonylglycinuria | Biotinidase deficiency
Trial Stage
Phase III
Drug Modality
Small molecule
Paediatric Trial
Yes

Key dates

Initial CTIS Submission Date
27-11-2024
First CTIS Authorization Date
12-03-2025

Trial design

Randomised, open-label, medebiotin fuerte solución inyectable (commercial comparator, active substance: biotin sodium; marketing authorisation es 34.236) and medebiotín fuerte comprimidos (commercial comparator, active substance: biotin; marketing authorisation es 24616). usual prescribed treatment vs biotin master formula (chewable formulation). maximum daily dose listed as 15 mg; treatment duration per arm 4 months then cross to other arm for 4 months (crossover).-controlled, crossover Phase III trial in Spain.

Randomised
Yes
Open Label
Yes
Comparator
Medebiotin Fuerte solución inyectable (commercial comparator, active substance: biotin sodium; marketing authorisation ES 34.236) and Medebiotín Fuerte Comprimidos (commercial comparator, active substance: biotin; marketing authorisation ES 24616). Usual prescribed treatment vs biotin master formula (chewable formulation). Maximum daily dose listed as 15 mg; treatment duration per arm 4 months then cross to other arm for 4 months (crossover).
Crossover
Yes
Target Sample Size
14
Trial Duration For Participant
240

Eligibility

Recruits 14 paediatric patients.

Vulnerable Population
Paediatric participants aged 3-18 years. Consent is provided by parent(s) or guardian(s). Assent documents are available for adolescents (assent forms for 12-17 years are listed). Separate subject information and informed consent forms for parents/guardians and adults are included (documents in Spanish).

Inclusion criteria

  • {"criterion_text":"- Outpatients of both sexes, between 3-18 years of age (both ages included within the range). Diagnosis of one of the two rare metabolic diseases under study: methylcrotonylglycinuria treated with Medebiotin tablets or injectable solution, biotinidase deficiency treated with Medebiotin tablets or injectable solution. The patient has no swallowing problems at the time of signing the informed consent document by the parent(s) or guardian(s). Good compliance with the treatment prescribed by the specialist for each patient during the last year (Medebiotin tablets or injectable solution). This implies that the patient has not presented any metabolic decompensation in the last year."}

Exclusion criteria

  • {"criterion_text":"- Known hypersensitivity to any of the excipients of the biotin master formulation. Any disorder or situation (severe metabolic decompensation) which, in the opinion of the investigating physician, would pose a risk for the subject not to comply with the prescribed treatment."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- visual scale and in the collection of subjective symptoms reported by the patient.","definition_or_measurement_approach":"Measured by visual scale and collection of subjective symptoms reported by the patient (as stated in endpoint text)."}

Secondary endpoints

  • {"endpoint_text":"- Determination of organic acids","definition_or_measurement_approach":"Laboratory determination of organic acids (as stated)."}
  • {"endpoint_text":"- plasma biotin levels","definition_or_measurement_approach":"Measurement of biotin concentration in plasma (as stated)."}

Recruitment

Planned Sample Size
14
Recruitment Window Months
9
Consent Approach
Informed consent obtained from parent(s) or guardian(s). Assent forms available for adolescents (assent documents for 12-17 years are listed). Subject information and informed consent forms for parents/guardians and adults are provided (documents in Spanish). Contact and consent documents versions are included in the submission (multiple ICF/assent documents).

Geography

Total Number Of Sites
1
Total Number Of Participants
14

Spain

Earliest CTIS Part Ii Submission Date
18-02-2025
Latest Decision Or Authorization Date
12-03-2025
Processing Time Days
22
Number Of Sites
1
Number Of Participants
14

Sites

Site Name
Complexo Hospitalario Universitario De Santiago
Department Name
Unidad de Enfermedades Metabólicas Congénitas
Principal Investigator Name
Maria Luz Couce Pico
Principal Investigator Email
Maria.Luz.Couce.Pico@sergas.es
Contact Person Name
Maria Luz Couce Pico
Contact Person Email
Maria.Luz.Couce.Pico@sergas.es
Number Of Participants
14

Sponsor

Primary sponsor

Full Name
Fundacion Publica Galega Instituto De Investigacion Sanitaria De Santiago De Compostela
Organisation Type
Hospital/Clinic/Other health care facility
Country Of Registered Address
Spain

Investigational products

Investigational Product Name
BIOTIN (chewable tablet)
Active Substance
Biotin
Modality
Small molecule
Routes Of Administration
Oral
Route
Oral
Authorisation Status
No marketing authorisation (marketingAuthNumber = '-')
Maximum Dose
15 mg
Investigational Product Name
Medebiotin Fuerte solución inyectable (SOLUTION FOR INJECTION)
Active Substance
Biotin sodium
Modality
Small molecule
Routes Of Administration
Oral
Route
Oral
Authorisation Status
Marketing authorisation ES 34.236
Maximum Dose
15 mg
Investigational Product Name
Medebiotín Fuerte Comprimidos (TABLET)
Active Substance
Biotin
Modality
Small molecule
Routes Of Administration
Oral
Route
Oral
Authorisation Status
Marketing authorisation ES 24616
Maximum Dose
15 mg

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