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Pharmacokinetics of Alglucosidase Alfa in Patients Aged 8 Years of Age andOlder

Phase 1
Conditions
Pompe disease (acid alpha-glucosidase deficiency)
MedDRA version: 18.1Level: LLTClassification code 10036143Term: Pompe's diseaseSystem Organ Class: 100000004850
Therapeutic area: Diseases [C] - Nutritional and Metabolic Diseases [C18]
Registration Number
EUCTR2010-022231-11-DE
Lead Sponsor
Genzyme Corporation
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
All
Target Recruitment
21
Inclusion Criteria

-A patient must meet all of the following criteria to be eligible for this study.
-The patient and/or the patient's parent/legal guardian is willing and
able to provide signed informed consent.
-The patient is =8 years of age with confirmed acid a-glucosidase (GAA) enzyme deficiency from skin, blood, or muscle tissue and/or 2 confirmed GAA gene mutations.
-The patient, if female and of childbearing potential, must have a
negative pregnancy test (urine beta-human chorionic gonadotropin) at baseline. Note: All female patients of childbearing potential and sexually mature males must agree to use a medically accepted method of contraception throughout the study.
-For patients previously treated with alglucosidase alfa the patient has received alglucosidase alfa for at least 6 months.
Are the trial subjects under 18? yes
Number of subjects for this age range: 10
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 10
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

-A patient who meets any of the following criteria will be excluded from this study.
-The patient is participating in another clinical study using an
investigational product.
-The patient, in the opinion of the Investigator, is unable to adhere to the requirements of the study

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: The primary objective of this study is to characterize the pharmacokinetics of alglucosidase alfa manufactured at the 4000 L scale in patients 8 years of age and older who have a confirmed diagnosis of Pompe disease.;Secondary Objective: A secondary objective of this study is to evaluate and explore the<br>relationship between anti-rhGAA antibody titers and the<br>pharmacokinetics of alglucosidase alfa..;Primary end point(s): Maximum observed concentration (Cmax)<br>Actual sampling time to reach maximum observed concentration (Tmax)<br>Area under the concentration-time curve from 0 to the time of the last quantifiable concentration (AUC last)<br>Area under the concentration-time curve from time 0 and extrapolated to infinite time (AUC inf)<br>Terminal elimination half-life (T1/2)<br>Total systemic clearance (CL)<br>Volume of distribution (Vd);Timepoint(s) of evaluation of this end point: Day 1
Secondary Outcome Measures
NameTimeMethod
Secondary end point(s): Pharmacokinetic (PK) parameters assessed in relation to anti-rhGAA<br>antibody titers<br>Pharmacokinetic (PK) parameters assessed in relation to<br>inhibitory/neutralizing anti-rhGAA antibody titers.;Timepoint(s) of evaluation of this end point: Day 1
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