Retinitis Pigmentosa
Conditions
Keywords
Retinitis pigmentosa, Ganglion cells, Inner retina, Optogenetics
Brief summary
The aim of this study is an explorative prospective observation of retinal ganglion cell degeneration and/or inner retina degeneration in Retinitis pigmentosa patients. The rate and patterns of progression will be correlated to the outer retina layers as well as clinical and genetic data of patients. Secondary goal is to determine potential parameters for optogenetic treatment eligibility.
Interventions
DIAGNOSTIC_TESTimaging
OCT, OCTA, RNFL-OCT, fundus autofluorescence, fundus imaging, flaremeter, fundus and slit lamp examination
Sponsors
University Medical Center Goettingen
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* genetically diagnosed Retintis pigmentosa * minimum age of 18 years (legal adult age in Germany) * patient consent for study participation
Exclusion criteria
* lack of capacity to consent in participation of study (unconsciousness, mental capacity, mental illness) * reduced cooperation during imaging or examination * age under 18 years * presence of additional retinal diseases * insufficient imaging quality due to hazy media (cornea, lense)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Thickness of ganglion cell inner plexiforme layer (GCIPL) in OCT imaging | 6 months intervals, overall time span 5 years | Measuring the thickness of the ganglion cell inner plexiforme layer (GCIPL) in macula optical coherence tomography (OCT) imaging of the retina. |
| Thickness of RNFL thickness in OCT imaging | 6 months intervals, overall time span 5 years | Measuring the thickness of the retinal nerve fiber layer (RNFL) in macular and optic nerve optical coherence tomography (OCT) imaging of the retina. |
| PD, VD, ICA of the SRP and DRP with OCTA imaging | 6 months intervals, overall time span 5 years | Perfusion density (PD), vessel density (VD), intercapillary area size (ICA) of superficial retinal plexus (SRP) and deep retinal plexus (DRP) in OCTA imaging (optical coherence tomography angiography) |
| Thickness and morphology of inner and outer retinal layers measured with OCT, fundus photography, FAF | 6-12 months intervals, overall time span 5 years | Thickness and morphology of inner and outer retinal layers measured with optical coherence tomography (OCT), fundus photography, fundus autofluorescence (FAF) |
| Changes in functional parameters such as BCVA | 6 months intervals, overall time span 5 years | Changes in functional parameters such as best corrected visual acuity |
| Correlations of outcome 1-5 with clinical data of patients | 6 months intervals, overall time span 5 years | Find correlations of imaging outcomes with the clinical data of patients, such as sex, age, age of onset of disease |
| Correlations of outcome 1-5 with genetic data of patients | 6 months intervals, overall time span 5 years | Find correlations of imaging outcomes with the Retinitis pigmentosa mutation and inheritance pattern of patients |
| Correlation of outcome 1-5 with inflammatory markers | 6 months intervals, overall time span 5 years | Correlation of imaging outcomes and disease progression with inflammatory markers collected with serum blood samples and flaremeter as well as slit lamp and fundus examination findings |
| Correlation of inner and outer retina parameters over time | 6 months intervals, overall time span 5 years | Correlation of changes in inner retina and outer retina of collected imaging data over time. |
Countries
Germany
Outcome results
None listed