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Cone photoreceptor structure and function in choroideremia assessed using adaptive optics scanning light ophthalmoscopy Peiluo Xu
- Format:
- Book
- Thesis/Dissertation
- Author/Creator:
- Xu, Peiluo, author.
- Language:
- English
- Subjects (All):
- Bioengineering.
- Ophthalmology.
- Health sciences.
- 0202.
- 0566.
- 0381.
- Local Subjects:
- Bioengineering.
- Ophthalmology.
- Health sciences.
- 0202.
- 0566.
- 0381.
- Genre:
- Academic theses
- Physical Description:
- 1 online resource (130 pages)
- Contained In:
- Dissertations Abstracts International 87-12B
- Place of Publication:
- Ann Arbor : ProQuest Dissertations and Theses, 2026
- Language Note:
- English
- Summary:
- Choroideremia (CHM) is an X-linked retinal degeneration causing loss of photoreceptors, retinal pigment epithelium, and choroid. A typical feature of CHM is a centrally maintained retina and RPE surrounded by peripheral areas of atrophic, dyspigmented retina. CHM patients usually retain visual acuity and sensitivity within this centrally retained island until late-stage disease. However, clinical measurements are limited by dynamic range and spatial resolution, and therefore, may not reflect subtle cellular-level changes in CHM. This thesis uses adaptive optics scanning light ophthalmoscopy (AOSLO) to achieve non-invasive in vivo imaging of the photoreceptor inner segment and waveguiding outer segment mosaics with single-cell resolution. Additionally, we have equipped our AOSLO with AO-guided microperimetry, which enables us to measure retinal threshold for small stimuli, and optoretinography (ORG), which captures physiological optical signals from photoreceptors in response to visible stimuli. On AOSLO images of CHM, patches of hyper- and hypo-reflective cones are commonly observed, even within the preserved central retina region. However, the extent to which local variations in cone reflectance correspond with changes in cone function remains unknown.This thesis examined the structure, ORG, and retinal sensitivity of individual or small patches of cones in conjunction in CHM patients compared with normal-sighted participants. We found differences in cone structure, as well as a decrease in ORG and sensitivity in CHM compared to normal-sighted participants across retinal eccentricity. We also discovered differences in ORG between hyper- and hypo-reflective cone patches while sensitivity was comparable. These findings contribute to the understanding of CHM disease mechanism on a cellular level and enable the development of sensitive biomarkers for assessing CHM disease state, which will prove helpful for early disease intervention and assessing treatment efficacy. Overall, these results indicate that retinal imaging with AOSLO and AO-based functional testing provide sensitive biomarkers for CHM, especially for early-stage disease, and may guide monitoring and therapeutic evaluation in CHM
- Notes:
- Source: Dissertations Abstracts International, Volume: 87-12, Section: B.
- Advisors: Morgan, Jessica I. W.; Brainard, David H. Committee members: Dunaief, Joshua L.; Yushkevich, Paul A.
- Ph.D. University of Pennsylvania 2026
- Vendor supplied data
- Local Notes:
- School code: 0175
- ISBN:
- 9798247981312
- Access Restriction:
- Restricted for use by site license
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