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Pharmacokinetics of Alglucosidase Alfa in Patients With Pompe Disease

Phase 4
Completed
Conditions
Pompe Disease
Glycogen Storage Disease Type II (GSD II)
Interventions
Biological: alglucosidase alfa
Registration Number
NCT01410890
Lead Sponsor
Genzyme, a Sanofi Company
Brief Summary

* The primary objective of this study was to characterize the pharmacokinetics (PK) of alglucosidase alfa manufactured at the 4000 L scale in participants who had a confirmed diagnosis of Pompe disease.

* A secondary objective of this study was to evaluate and explore the relationship between anti-recombinant human acid alpha-glucosidase antibody titers and the PK of alglucosidase alfa.

Detailed Description

The total study duration per participant was 4 to 8 weeks that consisted of a screening period (from 2 days to 4 weeks), treatment visit (1 day), and a follow up call (greater than or equal to 30 days). Two participants enrolled prior to protocol amendment 2 (dated 17 December 2015), which changed the study to single-dose, and were treated for 26 weeks, with a 4-week follow up.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
21
Inclusion Criteria

A participant was to meet all of the following criteria to be eligible for this study:

  • The participant and/or the participant's parent/legal guardian was willing and able to provide signed informed consent.
  • The participant had a confirmed acid alpha-glucosidase (GAA) enzyme deficiency from skin, blood, or muscle tissue and/or 2 confirmed GAA gene mutations.
  • Infant and toddler Pompe disease participants could be included in the study only under condition (minimal body weight) that the trial-related blood loss (including any losses in the maneuver) would not exceed 3 percent (%) of the total blood volume during a period of 4 weeks and would not exceed 1 % at any single time.
  • The participant, if female and of childbearing potential, must have had a negative pregnancy test (urine beta-human chorionic gonadotropin) at screening. Note: All female participants of childbearing potential and sexually mature males must have agreed to use a medically accepted method of contraception throughout the study.
  • For participants previously treated with alglucosidase alfa the participant had received alglucosidase alfa for at least 6 months.
Exclusion Criteria

A participant who met any of the following criteria was excluded from this study:

  • The participant was participating in another clinical study using an investigational product.
  • The participant, in the opinion of the Investigator, was unable to adhere to the requirements of the study.

The above information was not intended to contain all considerations relevant to a participant's potential participation in a clinical trial.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Alglucosidase alfaalglucosidase alfaParticipants received intravenous (IV) infusion of Alglucosidase alfa 20 milligrams per kilogram (mg/kg) body weight on Day 1. Infusion was administered at an initial rate of approximately 1 milligram per kilogram per hour (mg/kg/hr) with allowed rate increased of 2 mg/kg/hr every 30 minutes, if there were no signs of infusion-associated reactions (IARs), until a maximum rate of approximately 7 mg/kg/hr was reached.
Primary Outcome Measures
NameTimeMethod
Pharmacokinetics (PK): Maximum Observed Plasma Concentration (Cmax) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Cmax was defined as maximum observed plasma concentration.

Pharmacokinetics: Area Under the Concentration-time Curve From Time 0 to the Time of the Last Quantifiable Concentration (AUC0-last) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

AUC0-last was defined as area under the concentration-time curve from time 0 to the time of the last quantifiable concentration.

Pharmacokinetics: Time to Reach Maximum Observed Plasma Concentration (Tmax) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Tmax was defined as time to reach maximum observed plasma concentration.

Pharmacokinetics: Total Systemic Clearance (CL) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

CL of a drug is a measure of the rate at which a drug is metabolized or eliminated by normal biological processes.

Pharmacokinetics: Area Under the Plasma Concentration-Time Curve (AUC) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

AUC was defined as area under the plasma concentration-time curve from time 0 to 24 hours post-dose.

Pharmacokinetics: Terminal Elimination Half-life (T1/2) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

T1/2 was defined as the time taken by drug to reduce to half of its initial plasma concentration.

Pharmacokinetics: Volume of Distribution at Steady State (Vss) of Alglucosidase AlfaPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Volume of distribution (Vd) is defined as the theoretical volume in which the total amount of drug would need to be uniformly distributed to produce the desired plasma concentration of a drug. Vss is the apparent volume of distribution at steady-state.

Secondary Outcome Measures
NameTimeMethod
Pharmacokinetics: Area Under the Concentration-time Curve From Time 0 to the Time of the Last Quantifiable Concentration of Alglucosidase Alfa in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

AUC0-last was defined as area under the plasma concentration-time curve from time 0 to the time of the last quantifiable concentration.

Pharmacokinetics: Volume of Distribution in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Vd is defined as the theoretical volume in which the total amount of drug would need to be uniformly distributed to produce the desired plasma concentration of a drug. Vss is the apparent volume of distribution at steady-state.

Pharmacokinetics: Maximum Observed Plasma Concentration of Alglucosidase Alfa in Anti-Recombinant Human Acid Alpha-Glucosidase Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Cmax was defined as maximum observed plasma concentration.

Pharmacokinetics: Area Under the Concentration-time Curve From Time 0 and Extrapolated to Infinite Time (AUC0-inf) in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

AUC0-inf was defined as area under the concentration-time curve from time 0 extrapolated to infinite time.

Pharmacokinetics: Time to Reach Maximum Observed Plasma Concentration in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

Tmax was defined as time to reach maximum observed plasma concentration.

Pharmacokinetics: Terminal Elimination Half-life of Alglucosidase Alfa in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

T1/2 was defined as the time taken by drug to reduce to half of its initial plasma concentration.

Pharmacokinetics: Total Systemic Clearance in Anti-rhGAA Antibody Positive and Negative ParticipantsPre-dose and at 1, 2, 4, 8, 12, and 24 hours Post-dose on Day 1

CL of a drug is a measure of the rate at which a drug is metabolized or eliminated by normal biological processes.

Trial Locations

Locations (12)

Investigational Site Number 1028

🇧🇬

Sofia, Bulgaria

Investigational Site Number 356001

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New Delhi, India

Investigational Site Number 356002

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Vellore, India

Investigational Site Number 643001

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Moscow, Russian Federation

Investigational Site Number 643002

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Moscow, Russian Federation

Investigational Site Number 804001

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Kiev, Ukraine

Investigational Site Number 826002

🇬🇧

Salford, United Kingdom

Investigational Site Number 826003

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Birmingham, United Kingdom

Investigational Site Number 840005

🇺🇸

Salt Lake City, Utah, United States

Investigational Site Number 840008

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Valhalla, New York, United States

Investigational Site Number 840007

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Cincinnati, Ohio, United States

Investigational Site Number 840003

🇺🇸

Fairfax, Virginia, United States

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