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Phase 4 N=67 Treatment

A Study of the Safety and Efficacy of Fabrazyme in Patients With Fabry Disease

Fabry Disease

Enrolled (actual)
67
Serious AEs
46.3%
Results posted
Aug 2010
Primary outcome: Primary: Difference in Inverse Serum Creatinine Within Patients' Slopes Between the Placebo AGAL-008-00 (NCT00074984) and Fabrazyme AGAL02503 (NCT00081497) Periods — -0.044; -0.073 dL/mg/year — p=0.0130

Study Design & Population

Study type
Interventional
Phase
Phase 4
Interventions
agalsidase beta (Biological)
Age
Pediatric, Adult, Older Adult · 16+ yrs
Sex
All
Sponsor
Genzyme, a Sanofi Company
Primary completion
Sep 2005

Outcome Measures

OutcomeResultp-value
PRIMARY
Difference in Inverse Serum Creatinine Within Patients' Slopes Between the Placebo AGAL-008-00 (NCT00074984) and Fabrazyme AGAL02503 (NCT00081497) Periods
-0.044; -0.073 0.0130 sig
SECONDARY
Serum Creatinine at Pre-Fabrazyme and 6, 12, and 18 Months
1.7; 1.8; 1.9; 2.1
SECONDARY
Estimated Glomerular Filtration Rate (eGFR) at Pre-Fabrazyme and 6, 12, and 18 Months
53.1; 52.6; 50.4; 47.8
SECONDARY
Plasma Globotriaosylceramide (GL-3) (Normal Plasma GL-3 Level is ≤ 7.03 µg/mL) at Pre-Fabrazyme and 6, 12, and 18 Months
9.0; 4.8; 4.7; 4.6
SECONDARY
Proteinuria at Pre-Fabrazyme and 6, 12, and 18 Months
1.3; 1.1; 1.3; 1.3

Summary

People with Fabry Disease have an alteration in their genetic material (DNA) which causes a deficiency of the alpha-galactosidase A enzyme. Fabrazyme (agalsidase beta) is a drug that helps to break down and removes 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 analyzed the safety and efficacy of Fabrazyme in the treatment of patients with Fabry disease that previously participated in the AGAL-008-00 (NCT0074984) study.

Eligibility Criteria

Inclusion Criteria

  • Patients must have successfully completed the previous double-blind study AGAL-008-00 (NCT00074984)
  • Patients must provide written informed consent prior to study participation
  • Female patients of childbearing potential must have a negative pregnancy test prior to each dosing and all female patients must use a medically accepted form of contraception throughout the study

Exclusion Criteria

  • The patient was unable to complete AGAL-008-00 (NCT00074984)
  • The patient has undergone kidney transplantation or is currently on dialysis
  • The patient has diabetes mellitus or presence of confounding renal disease
  • The patient has a clinically significant organic disease or an unstable condition that precludes participation
  • The patient is unwilling to comply with the protocol requirements
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00081497). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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