RecruitingNCT02683512

GBE Deficiency (GSD IV and APBD) Natural History Study


Sponsor

Duke University

Enrollment

200 participants

Start Date

Dec 1, 2015

Study Type

OBSERVATIONAL

Conditions

Summary

Collection and review of clinical information related to glycogen branching enzyme (GBE) deficiency, diagnosed as Glycogen Storage Disease Type IV (GSD IV) or Adult Polyglucosan Body Disease (APBD generated during clinic visits.


Eligibility

Min Age: 0 YearsMax Age: 90 Years

Inclusion Criteria8

  • Diagnosis of GSD IV or APBD via:
  • Two variants in the GBE1 gene
  • Deficient GBE activity in liver, muscle, skin fibroblast or other tissue
  • One variant in GBE1 gene with evidence of disease that is pathogenic, per the clinician
  • Able to provide informed consent for self (adults) or affected individual (minor or adults with a legally authorized representative)
  • Able to provide consent for release of medical records
  • Pregnant women with a diagnosis of GSD IV or APBD will be included
  • Histology as confirmed by clinician

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Interventions

OTHERNo intervention

This is an observational study that consists of data abstraction from patient medical records.


Locations(1)

Duke University Medical Center

Durham, North Carolina, United States

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NCT02683512