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Clinical Trials/NCT02178969
NCT02178969
Completed
Not Applicable

A Prospective Short-term Study to Evaluate Methodologies for the Assessment of Disease Extent, Impact, and Wound Evolution in Patients With Dystrophic Epidermolysis Bullosa (DEB)

Shire10 sites in 7 countries29 target enrollmentStarted: June 24, 2014Last updated:

Overview

Phase
Not Applicable
Status
Completed
Sponsor
Shire
Enrollment
29
Locations
10
Primary Endpoint
Wound Surface Area (WSA) of patient- and investigator-selected wounds

Overview

Brief Summary

The purpose of this study is to better understand disease extent and to identify appropriate methodologies to evaluate (dystrophic epidermolysis bullosa) DEB in a quantitative and qualitative manner.

Study Design

Study Type
Observational
Observational Model
Cohort
Time Perspective
Prospective

Eligibility Criteria

Sex
All
Accepts Healthy Volunteers
No

Inclusion Criteria

  • Written informed consent is provided. Patients 18 years of age and older, and parent(s)/legal guardian(s) of patients younger than 18 years of age, must provide written informed consent prior to participating in the study; additionally, informed assent will be obtained from patients younger than 18 years of age as specified by local requirements.
  • Patient must have a documented diagnosis of DEB based on genetic analysis showing a mutation in COL7A1 or, in the opinion of the investigator, a confirmed diagnosis of DEB based on histologic criteria (antigen mapping or electron microscopy).
  • Patient must have at least 5 wounds that are suitable for imaging, in the opinion of the investigator, at the time of enrollment.
  • Patient is willing and able to undergo the protocol-specified procedures.

Exclusion Criteria

  • Patient has used or is currently using experimental treatment for DEB including, but not limited to, bone marrow transplantation, systemic immune suppression, or experimental procedures that involve live cells, with potential for systemic spread such as gene transfer, stem cell infusions, or other cell type injections such that, in the opinion of the investigator, inclusion poses an unacceptable risk to the patient or interpretation of these study data.
  • Patient has squamous cell carcinoma with evidence of locally invasive disease or distant metastases.
  • Patient is pregnant.

Outcomes

Primary Outcomes

Wound Surface Area (WSA) of patient- and investigator-selected wounds

Time Frame: Over 4 weeks

Wound Surface Area (WSA) as a percentage of Body Surface Area (BSA) in patients with DEB

Time Frame: Over 4 weeks

Secondary Outcomes

  • The number, severity and relationship to study procedures of adverse events (AEs) and serious AEs (SAEs)(Over 4 weeks)

Investigators

Sponsor
Shire
Sponsor Class
Industry
Responsible Party
Sponsor

Study Sites (10)

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