RecruitingNCT01694953

The Natural History Study of Mitochondrial NeuroGastroIntestinal Encephalopathy (MNGIE)

The Rare Disease Clinical Research Network Natural History Study of MNGIE


Sponsor

Columbia University

Enrollment

20 participants

Start Date

Feb 1, 2013

Study Type

OBSERVATIONAL

Conditions

Summary

This is a multi-center natural history study of Mitochondrial NeurogastroIntestinal Encephalopathy (MNGIE). Patients will be followed over time to assess clinical symptoms. The investigators hope to learn more about the disease of MNGIE as well as develop useful measures of disease status for use in future clinical trials. Additional clinical centers will be listed as they become available.


Eligibility

Min Age: 5 Years

Inclusion Criteria7

  • Thymidine Phosphorylase (TP) defect:
  • homozygous or
  • compound heterozygous mutations in the TYMP gene, and/or
  • TP enzyme activity of \<20% of normal.
  • Increased plasma Thd \> 3 micromole/L
  • Increased plasma dUrd \> 7.5 micromole/L
  • Age requirement of at least 5 years of age.

Exclusion Criteria4

  • Participation in an interventional (study medication or other experimental intervention) study (within 1 month of participation in this study).
  • Unable to travel to site for research visits.
  • Unwillingness to sign informed consent form.
  • Substance abuser

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Locations(1)

Columbia University

New York, New York, United States

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NCT01694953