Rimeporide in Patients With Duchenne Muscular Dystrophy

Exclusion Criteria

• •Patients with significant renal disease or impairment, with Glomerular Filtration Rate estimated using plasma cystatin C level using the Filler formula less than 90ml/min/1.73m2
• •Current or history of liver disease or impairment,
• •History of any significant medical disorder which may confound the interpretation of either efficacy or safety data e.g. inflammatory, coagulation disease, unstable cardiac or respiratory disease
• •Acute illness within 4 weeks of the first administration of study medication which may interfere with study assessments;
• •Significant change of dosage and/or dosing regimens for corticosteroids planned for the duration of study medication;
• •Use of beta blockers / and ACEI or ARB unless at stable dose for at least 3 months prior to baseline;
• •Use of Proton Pump Inhibitors unless at a stable dose for at least 3 months prior to baseline
• •Use of aldosterone antagonists (i.e. spironolactone, eplerenone) within 3 months prior to first administration of study medication;
• •Use of anticoagulants, antithrombotics or antiplatelet agents,
• •Use of antibiotics with predominant renal secretion (e.g., cephalosporins), immunosuppressive agents exception corticosteroids, continuous treatment with non-steroidal, anti-inflammatory drugs (NSAIDs), or lithium;
• •Previous treatment with idebenone or other forms of Coenzyme Q10 within 1 month of the first administration of study medication;
• •Previous treatment with investigational drugs within 4 weeks (or 7 half-life if longer than 4 weeks) of the first administration of study medication including placebo;
• •A baseline QTc\>450msec,or history of risk factors for torsades de pointes (eg, heart failure, hypokalaemia, family history of long QT syndrome);
• •LVEF≤ 45% at screening or within the past 6 months and/or history of acute heart failure;
• •Ventilator dependent;
• •Known individual hypersensitivity to any of the ingredients/excipients of the study medication;
• •Patients with specific contraindication to MRI (e.g.: metallic foreign body, claustrophobia, etc.).