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Brittle Cornea Syndrome and Ehlers-Danlos Syndrome (EDS) type VI are rare collagen-connective tissue disorders that predispose affected individuals to the development of perforated corneas from the mildest of eye trauma or even spontaneously. Clinical studies evaluating riboflavin-corneal crosslinking have found that it dramatically increases corneal rigidity. Given the success and safety of riboflavin crosslinking, the investigators believe that it can increase the corneal stability in patients affected these disseases, preventing perforation. It is furthermore possible, that riboflavin crosslinking will allow corneal transplants to successfully be performed on blind eyes that have already perforated and opacified. The purpose of the study is to determine whether corneal crosslinking can be safely performed on individuals with Brittle Cornea Syndrome or Ehlers-Danlos Syndrome type VI.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Riboflavin | Drug | 0.1%, applied every 5 minutes for 60 minutes | ||
| Kera-X | Device | 3 mW/cm2, to the central 7.5 mm of the cornea, for 30 minutes |
| Measure | Description | Time Frame |
|---|---|---|
| Adverse Events from Cornea Riboflavin Crosslinking in Brittle Cornea Syndrome or Ehlers Danlos Type VI | 3 Months |
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Inclusion Criteria:
Adult Patient with:
Genetic diagnosis of either Brittle Cornea Syndrome or EDS-VI, and
Either:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hadassah Medical Organization | Jerusalem | Israel |
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| ID | Term |
|---|---|
| C536192 | Brittle cornea syndrome 1 |
| C536198 | Ehlers-Danlos syndrome type 6 |
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| ID | Term |
|---|---|
| D012256 | Riboflavin |
| ID | Term |
|---|---|
| D005415 | Flavins |
| D011621 | Pteridines |
| D006574 | Heterocyclic Compounds, 2-Ring |
| D000072471 | Heterocyclic Compounds, Fused-Ring |
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| D006571 | Heterocyclic Compounds |
| D006575 | Heterocyclic Compounds, 3-Ring |
| D003067 | Coenzymes |
| D045762 | Enzymes and Coenzymes |
| D010860 | Pigments, Biological |
| D001685 | Biological Factors |