started out [0.001).:<0.0001) and at two years (p=0.0019). Fourteen patients (8 in Fabrazyme-treated and 6 in placebo) had skin biopsies at first infusion and final visit. All Fabrazyme-treated patients had capillary endothelium and deep vessel endothelium scores of zero at the final visit. Four (4) of 6 placebo patients had non-zero capillary endothelium scores (p=0.0150), and 6 of 6 had non-zero deep vessel endothelium scores (p=0.0003). Sixty-seven patients who participated in Study 2 were subsequently entered into an open-label extension study in which all patients received 1 mg/kg of Fabrazyme every two weeks for up to a maximum of 18 months. There was a statistically significant reduction in mean plasma GL-3 levels with durability in effect through the additional 18 months of treatment in the extension study from pretreatment baseline. Study 3 (Pediatric Study) was an open-label, uncontrolled, multi-national, multi-center study to evaluate safety, pharmacokinetics, and pharmacodynamics of Fabrazyme treatment in 16 pediatric patients with Fabry disease (14 males, 2 females), who were ages 8 to 16 years at first treatment. All patients received Fabrazyme 1 mg/kg every two weeks for up to 48 weeks. At baseline, all 14 males had elevated plasma GL-3 levels (i.e., > 7.03 µg/mL), whereas the two female patients had normal plasma GL-3 levels. Twelve of the 14 male patients, and no female patients, had GL-3 inclusions observed in the capillary endothelium on skin biopsies at baseline. At Weeks 24 and 48 of treatment, all 14 males had plasma GL-3 within the normal range. The 12 male patients with GL-3 inclusions in capillary endothelium at baseline achieved GL-3 inclusion scores of 0 at Weeks 24 and 48 of treatment. The two female patients plasma GL-3 levels remained normal through study Week 48. No new safety concerns were identified in pediatric patients in this study, and the overall safety and efficacy profile of Fabrazyme treatment in pediatric patients was found to be consistent with that seen in adults. Immunologic responses in pediatric patients may differ from those in adults, as IgG seroconversion in pediatric patients was associated with prolonged half-life concentrations of Fabrazyme, a phenomenon rarely observed in adult patients [see Clinical Pharmacology (12.3) , Adverse Reactions (6.2) , and Use in Specific Populations (8.4) ] . Study 4 was an open-label, re-challenge study to evaluate the safety of Fabrazyme treatment in patients who had a positive skin test to Fabrazyme or who had tested positive for Fabrazyme-specific IgE antibodies. In this study, six adult male patients, who had experienced multiple or recurrent infusion reactions during previous clinical trials with Fabrazyme, were re-challenged with Fabrazyme administered as a graded infusion, for up to 52 weeks of treatment [see Warnings and Precautions (5.4) ] . The initial two re-challenge doses of Fabrazyme were administered as a 0.5 mg/kg dose per week at an initial infusion rate of 0.01 mg/min for the first 30 minutes (1/25 th the usually recommended maximum infusion rate). The infusion rate was doubled every 30 minutes thereafter, as tolerated, for the remainder of the infusion up to a maximum rate of 0.25 mg/min. If the patient tolerated the infusion, the dose was increased to 1 mg/kg every two weeks (usually recommended dose), and the infusion rate was increased by slow titration upwards [see Dosage and Administration (2) ]. Four of the six patients treated in this study received at least 26 weeks of study medication, and two patients discontinued prematurely due to recurrent infusion reactions [see Warnings and Precautions (5.4) ] . How Supplied/Storage and Handling Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme 5 mg vials are supplied in single-use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap. 35 mg vial: NDC 58468-0040-1 5 mg vial: NDC 58468-0041-1 Refrigerate vials of Fabrazyme at 2° to 8°C (36° to 46°F). DO NOT USE Fabrazyme after the expiration date on the vial. Reconstituted and diluted solutions of Fabrazyme should be used immediately. This product contains no preservatives. If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2° to 8°C (36° to 46°F). Patient Counseling Information Patients should be informed that a Registry has been established in order to better understand the variability and progression of Fabry disease in the population as a whole and in women [see Use in Specific Populations (8.6) ], and to monitor and evaluate long-term treatment effects of Fabrazyme. The Registry will also monitor the effect of Fabrazyme on pregnant women and their offspring. Patients should be encouraged to participate and advised that their participation is voluntary and may involve long-term follow-up. For more information, visit www.fabryregistry.com or call (800) 745-4447. Fabrazyme is manufactured and distributed by: Genzyme Corporation 500 Kendall Street Cambridge, MA 02142 1-800-745-4447 (phone) U.S. License Number: 1596 Fabrazyme and Genzyme are registered trademarks of Genzyme Corporation. Package Carton Principal Display Panel 35 mg Carton Package contains one vial of Fabrazyme agalsidase beta 35mg Store Refrigerated At 2-8°C (36-46°F) Do Not Freeze or Shake Contains No Preservatives For Single Use Only No U.S. Standard of Potency See package insert for dosage and administration. Package Carton Principal Display Panel 5 mg Carton Package contains one vial of Fabrazyme agalsidase beta 5mg Store Refrigerated At 2-8°C (36-46°F) Do Not Freeze or Shake Contains No Preservatives For Single Use Only No U.S. Standard of Potency See package insert for dosage and administration. Fabrazyme is a registered trademark of Genzyme Corporation. Fabrazyme agalsidase beta injection, powder, lyophilized, for solution Product Information Product Type HUMAN PRESCRIPTION DRUG LABEL Item Code (Source) NDC:58468-0041 Route of Administration INTRAVENOUS DEA Schedule Active Ingredient/Active Moiety Ingredient Name Basis of Strength Strength AGALSIDASE BETA (AGALSIDASE BETA) AGALSIDASE BETA 5 mg in 1 mL Inactive Ingredients Ingredient Name Strength MANNITOL SODIUM PHOSPHATE, MONOBASIC, MONOHYDRATE SODIUM PHOSPHATE, DIBASIC, HEPTAHYDRATE Packaging # Item Code Package Description 1 NDC:58468-0041-1 1 mL in 1.0 VIAL, GLASS Marketing Information Marketing Category Application Number or Monograph Citation Marketing Start Date Marketing End Date BLA BLA103979 12/17/2008 Fabrazyme agalsidase beta injection, powder, lyophilized, for solution Product Information Product Type HUMAN PRESCRIPTION DRUG LABEL Item Code (Source) NDC:58468-0040 Route of Administration INTRAVENOUS DEA Schedule Active Ingredient/Active Moiety Ingredient Name Basis of Strength Strength AGALSIDASE BETA (AGALSIDASE BETA) AGALSIDASE BETA 5 mg in 1 mL Inactive Ingredients Ingredient Name Strength MANNITOL SODIUM PHOSPHATE, MONOBASIC, MONOHYDRATE SODIUM PHOSPHATE, DIBASIC, HEPTAHYDRATE Packaging # Item Code Package Description 1 NDC:58468-0040-1 1 mL in 1.0 VIAL, GLASS Marketing Information Marketing Category Application Number or Monograph Citation Marketing Start Date Marketing End Date BLA BLA103979 12/17/2008 Labeler - Genzyme Corporation (025322157) Establishment Name Address ID/FEI Operations Genzyme Corporation 926029653 API MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 968278916 API MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 050424395 LABEL(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Hospira, Inc. 030606222 MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 968278874 API MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 968278932 API MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 943130096 API MANUFACTURE(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 034378252 ANALYSIS(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Genzyme Corporation 968302658 ANALYSIS(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations BioReliance 175174176 ANALYSIS(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations BioReliance 505004556 ANALYSIS(58468-0040, 58468-0041) Establishment Name Address ID/FEI Operations Bionique Testing Laboratories 108684523 ANALYSIS(58468-0040, 58468-0041) Revised: 12/2012 Genzyme Corporation Next Interactions Print this page Add to My Med List More about Fabrazyme (agalsidase beta) Side Effects During Pregnancy Dosage Information Drug Interactions Support Group Pricing & Coupons En Español 0 Reviews Add your own review/rating Drug class: lysosomal enzymes Consumer resources Fabrazyme Fabrazyme (Advanced Reading) Professional resources Fabrazyme (AHFS Monograph) Related treatment guides Fabry Disease> ]} FEATURED: CAR-T Cell Therapy Overview Mechanism of Action KTE-C19 Studies KTE-C19 Cancer Targets Adverse Events Manufacturing Drug Status Rx Availability Prescription only B Pregnancy Category No proven risk in humans N/A CSA Schedule Not a controlled drug Approval History Drug history at FDA Manufacturers Genzyme Corporation Sanofi-Aventis U.S. LLC Drug Class Lysosomal enzymes Related Drugs lysosomal enzymes Lumizyme , Myozyme , Elaprase , Aldurazyme , cerliponase alfa , alglucosidase alfa Fabry Disease agalsidase beta , More... 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