Status:

COMPLETED

The Natural History of Infantile Globoid Cell Leukodystrophy

Lead Sponsor:

Zymenex A/S

Conditions:

Infantile Globoid Cell Leukodystrophy

Eligibility:

All Genders

Up to 2 years

Brief Summary

The purpose of this natural history study is to understand more about the progression of infantile Krabbe disease, a very rare genetic disease. There is very little published longitudinal data with on...

Detailed Description

Infantile globoid cell leukodystrophy (GLD, or Krabbe disease) is a rare, inherited degenerative disorder of the central and peripheral nervous systems. It is characterized by the presence of globoid ...

Eligibility Criteria

Inclusion

  • Informed consent obtained before any study-related activities. (Study-related activities are any procedure that would not have been performed during normal management of the subject)
  • The patient must have a documented diagnosis of infantile globoid cell leukodystrophy with galactocerebroside ß-galactosidase (GALC) activity \< 0.50 nmol/h/mg protein and evidence of two pathogenic mutations in the GALC gene must be confirmed after the baseline visit
  • The patient must have an age at the time of screening \< 2 years
  • The patient's parent(s) and/or legal guardian must have the ability to comply with the clinical protocol

Exclusion

  • History of hematopoietic stem cell transplantation
  • Presence of known clinically significant cardiovascular, hepatic, pulmonary or renal disease or other medical condition
  • Presence of major congenital abnormality
  • Any other medical condition or serious intercurrent illness, or extenuating circumstance that, in the opinion of the principal investigator, would preclude participation in the study
  • Use of any investigational product within 30 days prior to study enrollment or currently enrolled in another study which involves clinical investigations
  • The patient's parent(s) and/or legal guardian is unable to understand the nature, scope, and possible consequences of the study
  • Patient is unable to comply with the protocol, i.e. inability to return for follow-up evaluations or otherwise unlikely to complete the study as determined by the principal investigator

Key Trial Info

Start Date :

September 1 2009

Trial Type :

OBSERVATIONAL

Allocation :

ACTUAL

End Date :

July 1 2014

Estimated Enrollment :

6 Patients enrolled

Trial Details

Trial ID

NCT00983879

Start Date

September 1 2009

End Date

July 1 2014

Last Update

March 8 2018

Active Locations (1)

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Page 1 of 1 (1 locations)

1

Children's Hospital of Pittsburgh, 4401 One Children's Hospital Drive,4401 Penn Avenue

Pittsburgh, Pennsylvania, United States, 15224