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Phase 4 N=21 Treatment

Study of the Effect of Velaglucerase Alfa (VPRIV®) on Bone-related Pathology in Treatment-naïve Participants With Type 1 Gaucher Disease

Gaucher Disease

Enrolled (actual)
21
Serious AEs
9.5%
Results posted
Feb 2022
Primary outcome: Primary: Change From Baseline in Lumbar Spine Bone Mineral Density (BMD) Z-Score up to End of Study (EOS) (Week 103) — 0.17 Z-score

Study Design & Population

Study type
Interventional
Phase
Phase 4
Interventions
Velaglucerase alfa (Drug); Vitamin D (Dietary_supplement)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Shire
Primary completion
Nov 2020

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline in Lumbar Spine Bone Mineral Density (BMD) Z-Score up to End of Study (EOS) (Week 103)
0.17
SECONDARY
Change From Baseline in Lumbar Spine (LS) BMD Z-score at Week 51
0.02
SECONDARY
Change From Baseline in Lumbar Spine BMD at Week 51 and EOS (Week 103)
0.006; 0.011
SECONDARY
Change From Baseline in Total Bone Marrow Burden (BMB) Score at Week 51 and EOS (Week 103)
-3.0; -3.0
SECONDARY
Change From Baseline in Hemoglobin Concentration at Week 13, 25, 37, 51, 65, 77, 89, and EOS (Week 103)
0.532; 0.764; 0.765; 0.935; 1.029; 1.015
SECONDARY
Change From Baseline in Platelet Count at Week 13, 25, 37, 51, 65, 77, 89, and EOS (Week 103)
38.06; 53.24; 62.23; 79.66; 75.03; 87.19
SECONDARY
Change From Baseline in Normalized Liver Volume at Week 51 and EOS (Week 103)
-0.353; -0.447
SECONDARY
Change From Baseline in Normalized Spleen Volume at Week 51 and EOS (Week 103)
-0.443; -0.556
SECONDARY
Change From Baseline in Severity of Bone Pain at Week 51 and EOS (Week 103)
-2.750; -3.250
SECONDARY
Change From Baseline in Bone Pain Interference Score at Week 51 and EOS (Week 103)
-1.286; -4.429
SECONDARY
Change From Baseline in Overall Fatigue Measured by Brief Fatigue Inventory (BFI) at Week 51 and EOS (Week 103)
-0.111; 0.044
SECONDARY
Number of Participants With Shift in World Health Organization (WHO) BMD Classifications Based on LS T-Scores at Week 51 and EOS (Week 103)
0; 0; 0; 1; 6; 3
SECONDARY
Number of Participants With Treatment-Emergent Adverse Events (TEAEs)
21
SECONDARY
Number of Participants Who Developed Positive Anti-velaglucerase Alfa Antibody Status
1

Summary

The primary purpose of this study is to evaluate the effect of VPRIV therapy (60 units per kilogram [U/kg] every other week [EOW]) in treatment-naive participants with type 1 Gaucher disease on change from baseline in lumbar spine (LS) bone mineral density (BMD) Z-score as measured by dual energy x-ray absorptiometry (DXA) after 24 months of treatment.

Eligibility Criteria

Inclusion Criteria

  • The participant has a documented diagnosis of type 1 Gaucher disease, as documented by deficient GCB activity in leukocytes (whole blood only) or cultured skin fibroblasts. Diagnosis by only dry blood spot test is insufficient. Diagnosis may be based on results obtained prior to screening if documented in the participant's medical history.
  • Participants must have a LS BMD Z-score less than ( =) 18 and less than or equal to ( ] 10 and < 30 ng/mL) treat with 4000 IU vitamin D per day for 1 month and rescreen.
  • The participant has previously interrupted ERT for safety reasons.
  • The participant has had hypersensitivity to the active substance or to any of the excipients.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02574286). 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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