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A Phase 2, Open-Label, Single Dose Level, 24-Week Study to Evaluate the Safety, Tolerability, and Pharmacodynamics of AT1001 in Patients with Fabry Disease - Phase 2 study of AT1001 in Fabry Disease

Phase 1
Conditions
Fabry Disease
MedDRA version: 8.1 Level: LLT Classification code 10016016
Registration Number
EUCTR2005-004384-33-GB
Lead Sponsor
Amicus Therapeutics
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
Not specified
Target Recruitment
0
Inclusion Criteria

Patients must be adult males between 18 and 65 years of age (inclusive) and hemizygous for Fabry disease. Patients must have a confirmed diagnosis of Fabry disease with a documented missense gene mutation (individual or familial) and enhanceable enzyme activity (in vitro test: meeting specific criteria for enhancement). The patients must be naïve to ERT and other therapies, except for palliative therapies for the signs and symptoms of Fabry disease or must stop ERT for at least 30 weeks or up to 13 months. Patients must have end organ dysfunction, even minimal, demonstrated by either evidence of left ventricular hypertrophy documented by abnormal ECG and echocardiogram or by previous cardiac biopsy, or renal insufficiency documented by common clinical assessments such as creatinine and glomerular filtration rate or by renal biopsy. Patient agrees to be sexually abstinent or use a condom with spermicide when engaging in sexual activity during the course of the study and for a period of 30 days following their completion of the study. Patients must be previously untreated by ERT or substrate depletion for Fabry disease, or if ERT or other specific treatment for Fabry disease was administered, must stop ERT for at least 30 weeks or up to 13 months (in the judgment of the investigator, it must be considered safe for the patient to stop therapy for the duration of the study). Patients must be willing to undergo two renal and three skin biopsies. Patients must be willing and able to sign an informed consent form.

Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
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

The patient will be excluded from the study if: he has a history of organ transplant; there is evidence of significant disease other than Fabry disease (e.g., end-stage renal disease; heart disease [per clinical history, documented event, testing or class III/IV according to the New York Heart Association classification]; current diagnosis of cancer, except for basal cell carcinoma of the skin; diabetes (unless HbA1c <= 8); or neurological disease that impairs his ability to participate in the study); serum creatinine is greater than 2 on day -2; QTc interval is > 450 msec; pacemaker or other contraindication for MRI scanning; taking a medication prohibited by the protocol or any experimental therapy for any indication. Patients who participated in a clinical trial in the last 30 days. Patients who have any other condition which, in th opinion of the investigator would jeopardize the safety of the patient or impact the validity of the study results.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Secondary Outcome Measures
NameTimeMethod
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