NCT00074971

Brief Summary

People with Fabry disease have an alteration in their genetic material (DNA) which causes a deficiency of the a-galactosidase A enzyme. Fabrazyme is a drug that helps to breakdown and remove certain types of fatty substances called "glycolipids." These glycolipids are normally present within the body in most cells. In Fabry disease, glycolipids build up in various tissues such as the liver, kidney, skin, and blood vessels because a-galactosidase A is not present, or is present in small quantities. The build up of glycolipid ("globatriaosylceramide" or "GL-3") levels in these tissues in particular is thought to cause the clinical symptoms that are common to Fabry disease. This study will test the safety and efficacy of Fabrazyme in the treatment of patients with Fabry disease.

Trial Health

93
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
58

participants targeted

Target at below P25 for phase_3

Timeline
Completed

Started Oct 1999

Longer than P75 for phase_3

Geographic Reach
5 countries

20 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

October 1, 1999

Completed
4.2 years until next milestone

First Submitted

Initial submission to the registry

December 24, 2003

Completed
1 day until next milestone

First Posted

Study publicly available on registry

December 25, 2003

Completed
11 months until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2004

Completed
Last Updated

December 4, 2013

Status Verified

December 1, 2013

First QC Date

December 24, 2003

Last Update Submit

December 2, 2013

Conditions

Keywords

a-Galactosidase AaGALr-haGALFabryGL-3Fabrazyme

Outcome Measures

Primary Outcomes (2)

  • Safety and efficacy

  • Morphologic assessment of GL-3 inclusions in the capillary endothelium (vasculature) of the kidney

Secondary Outcomes (6)

  • Changes in McGill Pain Questionnaire

  • Autonomic status

  • Glomerular filtration

  • Functional assessment of urinary protein excretion Ophthalmic changes

  • SF-36 Health Survey

  • +1 more secondary outcomes

Interventions

Eligibility Criteria

Age16 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Patients must have successfully completed the previous double-blind study (AGAL-1-002-98)
  • Patients must provide written informed consent prior to study participation
  • Female patients must have a negative pregnancy test prior to each dosing and use a medically accepted method of contraception throughout the study

You may not qualify if:

  • Patient has undergone kidney transplant or is currently on dialysis
  • Patient is pregnant or lactating
  • Patient is unwilling to comply with the requirements of the protocol
  • Patient has a clinically significant organic disease (with the exception of symptoms related to Fabry disease), including clinically significant cardiovascular, hepatic, pulmonary, neurologic, or renal disease, or other medical condition, serious intercurrent illness, or extenuating circumstances that, in the opinion of the investigator, would preclude participation in the study

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (20)

Cedars-Sinai Medical Center

Los Angeles, California, 90048, United States

Location

University of California San Fransisco

San Francisco, California, 94143, United States

Location

Northwest Oncology & Hematology Associates

Coral Springs, Florida, 33065, United States

Location

Children's Memorial Hospital

Chicago, Illinois, 60614, United States

Location

University of Iowa Hospital and Clinics

Iowa City, Iowa, 52242, United States

Location

Greater Baltimore Medical Center

Baltimore, Maryland, 21204, United States

Location

Beth Israel Deaconess Medical Center

Boston, Massachusetts, 02215, United States

Location

Fetal Diagnostic and Imaging Center

Billings, Montana, 59101, United States

Location

University of New Mexico

Albuquerque, New Mexico, 87131, United States

Location

Mount Sinai School of Medicine

New York, New York, 10029, United States

Location

University of Rochester School of Medicine

Rochester, New York, 14642, United States

Location

Hart Family Practice

Hickory, North Carolina, 28601, United States

Location

Hematology/Oncology Associates of South Texas

San Antonio, Texas, 78229, United States

Location

University of Washington School of Medicine

Seattle, Washington, 98195, United States

Location

Hospital Edouard Herriot

Lyon, Cedex 03, France

Location

Hospital Europeen Georges Pompidou

Paris, Cedex 15, France

Location

Academisch Medisch Centrum

Amsterdam, 1105 AZ, Netherlands

Location

University of Puerto Rico

San Juan, 00935, Puerto Rico

Location

National Hospital for Neurology and Neurosurgery

London, WC1N 3BG, United Kingdom

Location

Hope Hospital

Manchester, M6 8HD, United Kingdom

Location

Related Publications (1)

  • Germain DP, Charrow J, Desnick RJ, Guffon N, Kempf J, Lachmann RH, Lemay R, Linthorst GE, Packman S, Scott CR, Waldek S, Warnock DG, Weinreb NJ, Wilcox WR. Ten-year outcome of enzyme replacement therapy with agalsidase beta in patients with Fabry disease. J Med Genet. 2015 May;52(5):353-8. doi: 10.1136/jmedgenet-2014-102797. Epub 2015 Mar 20.

MeSH Terms

Conditions

Fabry Disease

Interventions

agalsidase beta

Condition Hierarchy (Ancestors)

SphingolipidosesLysosomal Storage Diseases, Nervous SystemBrain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesCerebral Small Vessel DiseasesCerebrovascular DisordersVascular DiseasesCardiovascular DiseasesGenetic Diseases, X-LinkedGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolism, Inborn ErrorsLipidosesLipid Metabolism, Inborn ErrorsLysosomal Storage DiseasesMetabolic DiseasesNutritional and Metabolic DiseasesLipid Metabolism Disorders

Study Officials

  • Medical Monitor

    Genzyme Coorporation

    STUDY DIRECTOR

Study Design

Study Type
interventional
Phase
phase 3
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
INDUSTRY

Study Record Dates

First Submitted

December 24, 2003

First Posted

December 25, 2003

Study Start

October 1, 1999

Study Completion

December 1, 2004

Last Updated

December 4, 2013

Record last verified: 2013-12

Locations