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Genetic Study of Patients Suffering From Congenital Amaurosis of Leber or From an Early Severe Retinal Dystrophy

Not Applicable
Completed
Conditions
Amaurosis
Retinal Diseases
Registration Number
NCT00422721
Lead Sponsor
Nantes University Hospital
Brief Summary

Retinal dystrophies are responsible for numerous cases of blindness, and there are no therapeutic possibilities today. Gene therapy is efficient in a dog model concerning dystrophy linked to a mutation of the rpe65 gene. If such a therapy is to be considered for humans, it is urgent to select, at a national level, patients suffering from dystrophy linked to a mutation of the rpe65 gene. The systematic correlation of phenotype/genotype is an anatomical-functional approach, but it also identifies patients who may be potentially included in a future gene therapy study. Indeed, identification of people with a mutation of rpe65 is still insufficient in France (compared to other European countries) because of a lack of systemic genotyping of retinal dystrophy.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
360
Inclusion Criteria
  • Patients with clinical characteristics of amaurosis of Leber
  • Patients suffering from an early severe retinal dystrophy
  • Patients with social insurance
  • Patients with a consent form signed
Exclusion Criteria
  • Retinal dystrophy with autosomal dominant transmission
  • Retinal dystrophy occuring after 5 years of age
  • Syndromical retinal dystrophy with one or more systemic manifestations
  • Familial macular degeneration
  • Familial choroid dystrophy
  • Non-degenerative retinopathology

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Primary Outcome Measures
NameTimeMethod
Secondary Outcome Measures
NameTimeMethod

Trial Locations

Locations (1)

CHU de Nantes

🇫🇷

Nantes, France

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