NCT00299000

Brief Summary

The purpose of the study is to evaluate the safety and efficacy of two dose levels of Naglazyme in infants under the age of one year who have MPS VI by monitoring physical appearance, x-ray of the skeletal system and growth.

Trial Health

90
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
4

participants targeted

Target at below P25 for phase_4

Timeline
Completed

Started May 2006

Typical duration for phase_4

Geographic Reach
3 countries

4 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

March 2, 2006

Completed
4 days until next milestone

First Posted

Study publicly available on registry

March 6, 2006

Completed
2 months until next milestone

Study Start

First participant enrolled

May 1, 2006

Completed
2.9 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

April 1, 2009

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

April 1, 2009

Completed
1.5 years until next milestone

Results Posted

Study results publicly available

October 15, 2010

Completed
Last Updated

July 22, 2011

Status Verified

July 1, 2011

Enrollment Period

2.9 years

First QC Date

March 2, 2006

Results QC Date

May 7, 2010

Last Update Submit

July 19, 2011

Conditions

Outcome Measures

Primary Outcomes (3)

  • Change in Height

    52 weeks

  • Change in Weight

    52 weeks

  • Change in Haed Circumference

    52 weeks

Secondary Outcomes (1)

  • Change in Urinary Glycosaminoglycan Levels

    minimum 52 weeks of dosing

Study Arms (2)

Naglazyme, 1.0 mg/kg

OTHER

Dose comparison

Drug: Naglazyme

Naglazyme, 2.0 mg/kg

OTHER

Dose Comparison

Drug: Naglazyme

Interventions

Weekly infusion for minimum of 52 weeks. Naglazyme is diluted in sterile 0.9% sodium chloride solution

Also known as: recombinant human N-acetylgalactosamine 4-sulfatase, rh-arylsulfatase B, rhASB
Naglazyme, 1.0 mg/kgNaglazyme, 2.0 mg/kg

Eligibility Criteria

AgeUp to 1 Year
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17)

You may qualify if:

  • Signed informed consent by a parent or legal guardian
  • Parent or legal guardian willing and able to comply with all study procedures
  • Equal to or greater than 36 weeks estimated gestational age by physical exam at birth
  • Has a diagnosis of MPS VI based on a documented prenatal diagnosis or fibroblast or leukocyte N-acetylgalactosamine 4-sulfatase (ASB) enzyme activity level of less than 10% of the lower limit of the normal range of the measuring laboratory
  • Is less than one year of age
  • Has no evidence of skeletal dysplasia based on physical exam

You may not qualify if:

  • Parent of legal guardian perceived to be unreliable or unavailable for study participation
  • Use of any investigational drug within 30 days prior to screening, or requirement for any investigational agent prior to completion of all scheduled study assessments
  • Has concurrent disease or condition that would interfere with study participation or safety (i.e., has previously undergone hematopoietic stem cell transplantation such as bone marrow or cord blood transplantation, or major organ transplantation)
  • Any condition that, in the view of the principle investigator, renders the subject at high risk from treatment compliance and/or completing the study
  • Has known hypersensitivity to Naglazyme
  • Has previously received Naglazyme

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (4)

Children's Hospital Los Angeles

Los Angeles, California, 90027, United States

Location

Children's Hospital Oakland

Oakland, California, 94609, United States

Location

Hospital Femme Mere Enfant Centre

Lyon, 69677, France

Location

Hospital PediAtrico de Coimbra

Coimbra, 3000-076, Portugal

Location

Related Publications (2)

  • Garcia P, Phillips D, Johnson J, Martin K, Randolph LM, Rosenfeld H, Harmatz P. Long-term outcomes of patients with mucopolysaccharidosis VI treated with galsulfase enzyme replacement therapy since infancy. Mol Genet Metab. 2021 May;133(1):100-108. doi: 10.1016/j.ymgme.2021.03.006. Epub 2021 Mar 14.

  • Harmatz PR, Garcia P, Guffon N, Randolph LM, Shediac R, Braunlin E, Lachman RS, Decker C. Galsulfase (Naglazyme(R)) therapy in infants with mucopolysaccharidosis VI. J Inherit Metab Dis. 2014 Mar;37(2):277-87. doi: 10.1007/s10545-013-9654-7. Epub 2013 Oct 10.

Related Links

MeSH Terms

Conditions

Mucopolysaccharidosis VI

Interventions

galsulfase

Condition Hierarchy (Ancestors)

MucopolysaccharidosesCarbohydrate Metabolism, Inborn ErrorsMetabolism, Inborn ErrorsGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesLysosomal Storage DiseasesMucinosesConnective Tissue DiseasesSkin and Connective Tissue DiseasesMetabolic DiseasesNutritional and Metabolic Diseases

Limitations and Caveats

Given the small number of patients (4) represented in this study, the outcomes observed in this study may not reflect or predict outcomes observed by physicians in clinical practice.

Results Point of Contact

Title
Medical Information Services
Organization
BioMarin Pharmaceutical Inc.

Study Officials

  • Celeste Decker, MD

    BioMarin Pharmaceutical

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
OTHER
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 4
Allocation
RANDOMIZED
Masking
NONE
Purpose
PREVENTION
Intervention Model
PARALLEL
Sponsor Type
INDUSTRY

Study Record Dates

First Submitted

March 2, 2006

First Posted

March 6, 2006

Study Start

May 1, 2006

Primary Completion

April 1, 2009

Study Completion

April 1, 2009

Last Updated

July 22, 2011

Results First Posted

October 15, 2010

Record last verified: 2011-07

Locations