Status:

COMPLETED

Tamoxifen in Duchenne Muscular Dystrophy

Lead Sponsor:

University Hospital, Basel, Switzerland

Conditions:

Duchenne Muscular Dystrophy

Eligibility:

MALE

78-16 years

Phase:

PHASE3

Brief Summary

A randomised, double blind, placebo controlled, 48-week clinical trial with a core population (group A) of 79 ambulant 6.5 to 12 years old Duchenne's muscular dystrophy (DMD) patients that are under s...

Detailed Description

This is a 48-week multicentre, parallel, randomised, double-blind, placebo controlled phase 3 safety and efficacy trial. There are two treatment arms: Tamoxifen (verum) and placebo (control), with tre...

Eligibility Criteria

Inclusion

  • Group A (ambulant patients)
  • Documented diagnosis of DMD by mutation analysis in the dystrophin gene or by substantially reduced levels of dystrophin protein (i.e. absent or \<5% of normal) on Western blot or immunostaining
  • Stable treatment with glucocorticoids \>6 months (no significant change in dosage (\>0.2mg/kg)) at screening; dosing adaptations according to weight change are allowed
  • Male gender
  • 5 to 12 years of age at time of screening
  • weight \>20kg
  • ambulant patients
  • able to walk at least 350 meters in 6 minute walking distance test without assistance at screening
  • MFM D1 subdomain of the MFM scale \>40% at screening
  • Ability to provide informed consent and to comply with study requirements
  • Patients harbouring a nonsense mutation treatable with the approved drug ataluren should be under stable ataluren treatment for at least 3 months or in case of nontolerance being off ataluren treatment for at least 3 months before screening
  • Group B (non-ambulant patients)
  • Documented diagnosis of DMD by mutation analysis in the dystrophin gene or by substantially reduced levels of dystrophin protein (i.e. absent or \<5% of normal) on Western blot or immunostaining
  • Not using glucocorticoids for \>6 months
  • Male gender
  • Non-ambulant patients (walking distance less than 10 meters)
  • 10 to 16 years of age at time of screening
  • Ability to provide informed consent and to comply with study requirements
  • Open label extension
  • \- Recent participation and completion of TAMDMD study

Exclusion

  • Known individual hypersensitivity or allergy to tamoxifen or other ingredients/excipients of IMP
  • Female gender
  • Use of tamoxifen or testosterone within the last 3 months
  • Known or suspected malignancy
  • Other chronic disease or clinically relevant limitation of renal, liver or heart function
  • Known or suspected non-compliance
  • Any injury which may impact functional testing, e.g. upper or lower limb fracture
  • Planned or expected spinal fusion surgery during the study period (as judged by the Investigator; i.e. due to rapid progressing scoliosis), previous spinal fusion surgery is allowed if it took place more than 6 months prior to screening.
  • Inability to follow the procedures of the study, e.g. due to language problems, psychological disorders of the participant/parents (as judged by the investigator)
  • Concomitant participation in any other interventional trial (and up to 3 months prior to screening)
  • Use of CYP2D6 inhibitors or of CYP3A4 inducers (apart from glucocorticoids), platelet aggregation inhibitors and coumarin-type anti-coagulants
  • Use of drugs metabolized by CYP2C9, such as phenprocoumon, phenytoin, warfarin, celecoxib, fluvastatin, ginko biloba, St. John's wort and sulfamethoxazol
  • Galactosemia (lack of galactose-1-phosphat-uridylyltransferase or UDP-galactose-4-epimerase or galactokinase; Fanconi-Bickel-syndrome); congenital lack of lactase; glucose-galactose malabsorption
  • Presence of one or more of the following eye disorders: cataract, retinopathia, optic neuropathy, alteration of the cornea
  • Presence of one or more of the following laboratory abnormalities: anaemia, thrombocytopenia, leukopenia, neutropenia or agranulocytosis
  • Group A:
  • Glucocorticoid naïve patients
  • Start of glucocorticoid treatment or change in dosage \<6 month prior to screening (dosing adaptations according to weight change are allowed)
  • Group B:
  • Glucocorticoid treated patients or patients that stopped glucocorticoid treatment \<6 month prior to screening
  • Assisted ventilation of any kind necessary

Key Trial Info

Start Date :

June 12 2018

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

October 18 2022

Estimated Enrollment :

93 Patients enrolled

Trial Details

Trial ID

NCT03354039

Start Date

June 12 2018

End Date

October 18 2022

Last Update

December 20 2022

Active Locations (11)

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Page 1 of 3 (11 locations)

1

Hôpitaux Raymond Poincaré

Garches, France, 92380

2

Hôpital de Hautepierre

Strasbourg, France, 67098

3

DRK Klinik Berlin Westend

Berlin, Germany, 14050

4

Universitätsklinikum Essen

Essen, Germany, 45147