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Efficacy and tolerability of ibuprofen and isosorbide dinitrate (20 mg, 40 mg, 60 mg, 80 mg)

Conditions
Therapeutic area: Body processes [G] - Genetic Phenomena [G05]
Restrictive respiratory syndrome with frequent pulmonary infections and cardiomyopathy.wasting of skeletal muscle, severe local inflammation and, at least initially, muscle regeneration.
MedDRA version: 17.0Level: PTClassification code 10064571Term: Gene mutationSystem Organ Class: 10010331 - Congenital, familial and genetic disorders
Registration Number
EUCTR2013-004427-37-IT
Lead Sponsor
PARENT PROJECT ONLUS
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Authorised-recruitment may be ongoing or finished
Sex
Male
Target Recruitment
188
Inclusion Criteria

1. 1.Patient written informed assent and parents/guardians written informed consent: provision of consent to participate in the study as shown by the signature on the Volunteer Consent Form after verbal and written information
2.Confirmed diagnosis of Duchenne muscular dystrophy (muscle biopsy, genetic and biochemical analysis)
3.Age =10 years and < 18 years
4.Patients non-ambulant (at least 1 year in a wheelchair) within the last 6 years
5.Patients who receive the standard of care for Duchenne muscular dystrophy as recommended by the Duchenne muscular dystrophy care recommendations
6.Patients on chronic glucocorticosteroid treatment: dosage must be stable for at least 6 months prior to the admission to the study

Are the trial subjects under 18? yes
Number of subjects for this age range: 188
F.1.2 Adults (18-64 years) no
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

1. Any acute co-morbid condition interfering with the well-being of the patient within 7 days of enrolment (including bacterial infections, viral infectious processes, food poisoning, temperature > 37° C). Patients will be excluded from enrolment until the infection had been appropriately treated and resolved;
2. any gastrointestinal conditions which, in the opinion of the Investigator, may interfere with the absorption of the drug or render the subjects unable to take oral medication (gastric ulcer, peptic ulcer, stomach acid, frequent diarrhoea, gastrointestinal surgery);
3. a left ventricular ejection fraction (EF) of < 30%;
4. forced vital capacity (FVC) < 40% of predicted;
5. history of recurrent headache;
6. history or presence of allergy or intolerance to the study drugs or their components or drugs of their class, or a history of drug or other allergy that, in the opinion of the responsible physician, contraindicates their participation;
7. inability to take tablets, as assessed by the Investigator by the end of the screening period;
8. previous or ongoing medical conditions, medical history, physical findings or laboratory abnormalities that could affect safety, make it unlikely that treatment and follow up will be correctly completed or impair the assessment of study results, in the judgment of the Investigator;
9. weight of less than 13 kilograms;
10. participation in any other investigational drug or therapy study within the previous 6 months.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: To assess the efficacy of the combination of ibuprofen and isosorbide dinitrate (ISDN) in delaying the worsening of the muscular motor function in patients with Duchenne muscular dystrophy assessed by the Performance of Upper Limb (PUL) scale. ;Secondary Objective: To assess the safety and tolerability of the combination of ibuprofen and ISDN in Duchenne muscular dystrophy affected patients. <br>To assess the efficacy of the combination of ibuprofen and ISDN assessed by other muscular, cardiac and pulmonary measures of functionality and also its impact on quality of life<br>;Primary end point(s): The primary outcome will be the mean change (%) from baseline to the end of the study in the MFM score;Timepoint(s) of evaluation of this end point: Treatment period: 18 months
Secondary Outcome Measures
NameTimeMethod
Secondary end point(s): Secondary outcomes include other muscular measures, cardiac and pulmonary function and Quality of Life. ;Timepoint(s) of evaluation of this end point: 18 months
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