Trial Outcomes & Findings for A Study Evaluating Glycosphingolipid Clearance in Patients Treated With Agalsidase Alfa Who Switch to Agalsidase Beta (NCT NCT01650779)

NCT ID: NCT01650779

Last Updated: 2014-07-10

Results Overview

Percent change from baseline = (\[post-baseline value minus baseline value\] divided by \[baseline value\]) multiplied by 100. For levels reported as below quantitative limit (BQL), the lower limit of quantitation (LLOQ) value was divided by 2 and used in the calculation to estimate values in samples that were BQL. The LLOQ for plasma lyso-GL-3 was 5.0 nanogram per milliliter (ng/mL). This study is exploratory because little is known about the dose-response of these biomarkers to enzyme replacement therapy (ERT) or about the clinical significance of the biomarkers.

Recruitment status

COMPLETED

Study phase

PHASE4

Target enrollment

15 participants

Primary outcome timeframe

Baseline, Month 2, 4, 6

Results posted on

2014-07-10

Participant Flow

The study was conducted at 6 centers in the United States of America between April 30, 2012 and March 15, 2013.

A total of 16 participants were screened of which 1 participant was screen failure. A total of 15 participants were enrolled in this study.

Participant milestones

Participant milestones
Measure
Agalsidase Beta
Commercially available agalsidase beta (Fabrazyme ®) 1.0 milligram per kilogram (mg/kg) administered as an intravenous infusion every 2 weeks (q2w) up to Month 6.
Overall Study
STARTED
15
Overall Study
COMPLETED
14
Overall Study
NOT COMPLETED
1

Reasons for withdrawal

Reasons for withdrawal
Measure
Agalsidase Beta
Commercially available agalsidase beta (Fabrazyme ®) 1.0 milligram per kilogram (mg/kg) administered as an intravenous infusion every 2 weeks (q2w) up to Month 6.
Overall Study
Withdrawal by Subject
1

Baseline Characteristics

A Study Evaluating Glycosphingolipid Clearance in Patients Treated With Agalsidase Alfa Who Switch to Agalsidase Beta

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Agalsidase Beta
n=15 Participants
Commercially available agalsidase beta 1.0 mg/kg administered as an intravenous infusion q2w up to Month 6.
Age, Continuous
28.5 years
STANDARD_DEVIATION 16.11 • n=99 Participants
Sex: Female, Male
Female
0 Participants
n=99 Participants
Sex: Female, Male
Male
15 Participants
n=99 Participants
Race/Ethnicity, Customized
Caucasian
13 participants
n=99 Participants
Race/Ethnicity, Customized
Hispanic
1 participants
n=99 Participants
Race/Ethnicity, Customized
Other
1 participants
n=99 Participants
Duration of Fabry Disease
9.3 years
FULL_RANGE 4.73 • n=99 Participants
Method of Diagnosis of Fabry Disease
Genotype
14 participants
n=99 Participants
Method of Diagnosis of Fabry Disease
Leukocyte alfa-galactosidase A (GAL) activity
11 participants
n=99 Participants
Method of Diagnosis of Fabry Disease
Plasma alfa-GAL activity
9 participants
n=99 Participants

PRIMARY outcome

Timeframe: Baseline, Month 2, 4, 6

Population: All enrolled participants were included in the analysis. Here, 'n' signifies participants with plasma Lyso-GL-3 assessment at the specified time point.

Percent change from baseline = (\[post-baseline value minus baseline value\] divided by \[baseline value\]) multiplied by 100. For levels reported as below quantitative limit (BQL), the lower limit of quantitation (LLOQ) value was divided by 2 and used in the calculation to estimate values in samples that were BQL. The LLOQ for plasma lyso-GL-3 was 5.0 nanogram per milliliter (ng/mL). This study is exploratory because little is known about the dose-response of these biomarkers to enzyme replacement therapy (ERT) or about the clinical significance of the biomarkers.

Outcome measures

Outcome measures
Measure
Agalsidase Beta
n=15 Participants
Commercially available agalsidase beta 1.0 mg/kg administered as an intravenous infusion q2w up to Month 6.
Percent Change From Baseline in Plasma Deacylated Globotriaosylceramide (Lyso-GL-3) at Month 2, 4 and 6
Month 2 (n=14)
-31.71 percent change
Standard Deviation 22.540
Percent Change From Baseline in Plasma Deacylated Globotriaosylceramide (Lyso-GL-3) at Month 2, 4 and 6
Month 4 (n=12)
-39.04 percent change
Standard Deviation 22.280
Percent Change From Baseline in Plasma Deacylated Globotriaosylceramide (Lyso-GL-3) at Month 2, 4 and 6
Month 6 (n=14)
-39.54 percent change
Standard Deviation 23.567

SECONDARY outcome

Timeframe: Baseline, Month 2, 4, 6

Population: All enrolled participants were included in the analysis. Here, 'n' signifies participants with plasma GL-3 assessment at the specified time point.

Percent change from baseline = (\[post-baseline value minus baseline value\] divided by \[baseline value\]) multiplied by 100. For levels reported as BQL, the LLOQ value was divided by 2 and used in the calculation to estimate values in samples that were BQL. The LLOQ for plasma GL-3 was 2.0 microgram per milliliter (mcg/mL). This study is exploratory because little is known about the dose-response of these biomarkers to ERT or about the clinical significance of the biomarkers.

Outcome measures

Outcome measures
Measure
Agalsidase Beta
n=15 Participants
Commercially available agalsidase beta 1.0 mg/kg administered as an intravenous infusion q2w up to Month 6.
Percent Change From Baseline in Plasma Globotriaosylceramide (GL-3) at Month 2, 4 and 6
Month 4 (n=10)
-12.80 percent change
Standard Deviation 23.388
Percent Change From Baseline in Plasma Globotriaosylceramide (GL-3) at Month 2, 4 and 6
Month 6 (n=12)
-17.89 percent change
Standard Deviation 25.291
Percent Change From Baseline in Plasma Globotriaosylceramide (GL-3) at Month 2, 4 and 6
Month 2 (n=12)
-10.33 percent change
Standard Deviation 21.087

SECONDARY outcome

Timeframe: Baseline, Month 2, 4, 6

Population: All enrolled participants were included in the analysis. Here, 'n' signifies participants with urine GL-3 assessment at the specified time point.

Percent change from baseline = (\[post-baseline value minus baseline value\] divided by \[baseline value\]) multiplied by 100. For levels reported as BQL, the LLOQ value was divided by 2 and used in the calculation to estimate values in samples that were BQL. The LLOQ for urine GL-3 was 0.2 mcg/mL. The absolute values were calculated in microgram per millimole (mcg/mmol) of creatinine by dividing GL-3 (mcg/mL) by creatinine (mg/mL) and multiplying by 113.13 (mg/mmol), the molecular weight of creatinine. For levels reported BQL, the absolute values were calculated in microgram per millimole (mcg/mmol) of creatinine by dividing 0.1 (mcg/mL) by creatinine (mg/mL) and multiplying by 133.13 (mg/mmol). This study is exploratory because little is known about the dose-response of these biomarkers to ERT or about the clinical significance of the biomarkers.

Outcome measures

Outcome measures
Measure
Agalsidase Beta
n=15 Participants
Commercially available agalsidase beta 1.0 mg/kg administered as an intravenous infusion q2w up to Month 6.
Percent Change From Baseline in Urine GL-3 at Month 2, 4, and 6
Month 2 (n=13)
-44.71 percent change
Interval -98.4 to 1068.2
Percent Change From Baseline in Urine GL-3 at Month 2, 4, and 6
Month 4 (n=12)
-41.49 percent change
Interval -96.5 to 464.9
Percent Change From Baseline in Urine GL-3 at Month 2, 4, and 6
Month 6 (n=12)
-33.75 percent change
Interval -97.1 to 1116.7

SECONDARY outcome

Timeframe: Baseline, Month 2, 4, 6

Population: The data for this outcome measure was exploratory and to be collected in individual participant listing only. Analysis of this data was planned only if baseline data was collected on a large number of enrolled participants. This was not the case and therefore interpretation of these results were not possible.

Gastrointestinal symptoms (abdominal pain, abdominal distention, and irregular bowel movements) were to be assessed by a modified version of the Irritable Bowel Syndrome (IBS) Severity Scoring System. The modified IBS Severity Scoring System is a 7-item questionnaire. The severity score calculated by summing the scores of 5 of the 7 questions. Each of the 5 questions were scored on a scale of 0 to 100, leading to a total possible score range of 0 to 500, where higher scores indicate more severe gastrointestinal symptoms. The data for this outcome measure was exploratory and to be collected in individual participant listing only.

Outcome measures

Outcome data not reported

Adverse Events

Agalsidase Beta

Serious events: 0 serious events
Other events: 1 other events
Deaths: 0 deaths

Serious adverse events

Adverse event data not reported

Other adverse events

Other adverse events
Measure
Agalsidase Beta
n=15 participants at risk
Commercially available agalsidase beta 1.0 mg/kg administered as an intravenous infusion every 2 weeks up to Month 6.
General disorders
Infusion-associated reactions
6.7%
1/15 • Baseline up to end of study (Month 6) or early withdrawal
All enrolled participants were included in the analysis.

Additional Information

Trial Transparency Team

Sanofi

Results disclosure agreements

  • Principal investigator is a sponsor employee If no publication has occurred within 12 months of the completion of the study, the Investigator shall have the right to publish/present independently the results of the study. The Investigator shall provide the Sponsor with a copy of any such presentation/publication for comment at least 30 days before any presentation/submission for publication. If requested by the Sponsor, any presentation/submission shall be delayed up to 90 days, to allow the Sponsor to preserve its proprietary rights.
  • Publication restrictions are in place

Restriction type: OTHER