Topography of Slowed Dark Adaptation in Pseudoxanthoma Elasticum: PROPXE Study Report 1
Patients with PXE exhibit significant slowing of both cone- and rod-mediated dark adaptation, particularly in regions prone to BrM calcification. These findings suggest that dark adaptometry and assessment of BrM calcification can serve as clinical tools for evaluating disease severity and monitoring progression in PXE, enabling earlier interventions before the onset of exudative MNV or atrophy.
Authors
- Carlo Rivolta (ORCID: https://orcid.org/0000-0002-0733-9950)
- Sandra Liakopoulos (ORCID: https://orcid.org/0000-0003-3539-4372)
- Maximilian Pfau (ORCID: https://orcid.org/0000-0001-9761-9640)
- Nicolas Feltgen (ORCID: https://orcid.org/0000-0002-6857-3003)
- Mayss Al‐Sheikh (ORCID: https://orcid.org/0000-0002-3364-2232)
- Georg Ansari (ORCID: https://orcid.org/0009-0008-4693-2061)
- Sharon F. Terry (ORCID: https://orcid.org/0000-0002-0452-9329)
- Karolina Kamińska (ORCID: https://orcid.org/0000-0002-4720-5527)
- Chantal Dysli (ORCID: https://orcid.org/0000-0002-3926-0825)
- Kristina Pfau (ORCID: https://orcid.org/0000-0002-3642-3502)
- Mathieu Quinodoz (ORCID: https://orcid.org/0000-0002-9841-4433)
- Francesca Cancellieri (ORCID: https://orcid.org/0000-0001-8476-5366)
- Justus G. Garweg (ORCID: https://orcid.org/0000-0003-3973-5082)
- Jana Burghaus-Zhang
- Stephan Michels (ORCID: https://orcid.org/0000-0002-3146-7805)
Institutions
- Goethe University Frankfurt (DE)
- University of Bern (CH)
- University of Bonn (DE)
- University of Leicester (GB)
- University of Cologne (DE)
- University of Basel (CH)
- University of Zurich (CH)
- Heidelberg University (DE)
- University Hospital of Bern (CH)
- University Hospital Heidelberg (DE)
- Berner Augenklinik am Lindenhofspital (CH)
- Stadtspital Waid (CH)
- University Hospital Cologne (DE)
- Institute of Molecular and Clinical Ophthalmology Basel (CH)
Publication Details
- Journal
- Investigative Ophthalmology & Visual Science
- Published
- 2025-02-06
- DOI
- https://doi.org/10.1167/iovs.66.2.17
- Citations
- 6
- Primary Topic
- Dermatological and Skeletal Disorders
- Type
- article
- Field-Weighted Citation Impact
- 10.90
Funders
- Bayer
- Novartis
- Sanofi
- Biogen
- Apellis Pharmaceuticals
- Astellas Pharma
- Heidelberg Engineering
- National Institutes of Health
- Genentech
- Daiichi Sankyo Europe