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Phase 4 N=11 Randomized Quadruple-blind Treatment

A Safety and Efficacy Study of Two Dose Levels of Taliglucerase Alfa in Pediatric Subjects With Gaucher Disease

Gaucher Disease

Enrolled (actual)
11
Serious AEs
9.1%
Results posted
Nov 2014
Primary outcome: Primary: Hemoglobin — 1.2; 0.4; 1.1; 1.5 g/dL

Study Design & Population

Study type
Interventional
Phase
Phase 4
Interventions
Taliglucerase alfa (Drug)
Age
Pediatric · 2+ yrs
Sex
All
Sponsor
Pfizer
Primary completion
May 2012

Outcome Measures

OutcomeResultp-value
PRIMARY
Hemoglobin
1.2; 0.4; 1.1; 1.5; 0.9; 1.5
SECONDARY
Chitotriosidase
-32.4; -32.7; -49.6; -50.2; -57.2; -58.9
SECONDARY
Spleen Volume
1218; 1023; 811.6; 524.0
SECONDARY
Platelet Count
162667; 99600; 208167; 172200
SECONDARY
Chemokine (C-C Motif) Ligand 18 (CCL18)
-31.4; -18.3; -45.7; -37.3; -49.9; -46.5
SECONDARY
Liver Volume
1214; 991.7; 1116; 849.1

Summary

This is a multi-center, double-blind trial to assess the safety and efficacy of taliglucerase alfa in untreated subjects (2 to <18 years old) with Gaucher disease randomly assigned to treatment with one of two doses, 30 or 60 units/kg. Subjects will receive an intravenous (IV) infusion of taliglucerase alfa every two weeks. The total duration of treatment will be 12 months. At the end of the 12-month treatment period eligible subjects will be offered enrollment in an open-label extension study if taliglucerase alfa is not commercially available.

Eligibility Criteria

Inclusion Criteria

  • Males and females 2 to <18 years old.
  • Diagnosis of Gaucher disease with leukocyte acid β-glucosidase activity ≤30% of the mean of the reference range for healthy subjects.
  • Subjects who have not received enzyme replacement therapy (ERT) in the past or who have not received ERT in the past 12 months and have a negative anti-glucocerebrosidase antibody assay.
  • Subjects who have not received substrate reduction therapy (SRT) in the past 12 months.
  • Subjects whose clinical condition, in the opinion of the investigator, requires treatment with enzyme replacement therapy (ERT).

Exclusion Criteria

  • Currently taking another investigational drug for any condition.
  • Presence of neurological signs and symptoms characteristic of Gaucher disease with complex neuronopathic features other than longstanding oculomotor gaze palsy.
  • Presence of unresolved anemia due to iron, folic acid, or vitamin B12 deficiency
  • Previous hypersensitivity reaction to Cerezyme® (imiglucerase) or Ceredase® (alglucerase).
  • History of allergy to carrots.
  • Presence of HIV, HBsAg or hepatitis C infections.
  • Subject's parent(s) or legal guardian(s) are unable to understand the nature, scope and possible consequences of the study.
  • Presence of any medical, emotional, behavioral or psychological condition that in the judgment of the Investigator would interfere with the subject's compliance with the requirements of the study.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01132690). 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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