Assessing Residual Cone Function in Retinitis Pigmentosa Patients

作者: Tasneem A. Arsiwalla , Elisa E. Cornish , Phuc Vuong Nguyen , Maria Korsakova , Haipha Ali

DOI: 10.1167/TVST.9.13.29

关键词: Retinitis pigmentosaIn patientDisease progressionVisual acuityResidualFull fieldClinical recordMean ageOphthalmologyMedicine

摘要: Purpose The purpose of this study was to investigate cone function deterioration in patients with retinitis pigmentosa (RP) using full field electroretinogram (ffERG), pattern (pERG), and optical coherence tomography (OCT) their correlation visual acuity (VA). Methods Clinical records (2008-2018) RP undergoing repeat electrophysiology were reviewed. Results ffERG (30 Hz flicker fused amplitude [FFAmp]), pERG [p50 n95], macular OCT (ellipsoid zone [EZ] outer segment thickness) collected. One hundred twenty-six eyes from 63 (33 women, mean age 35 years) included. decline VA 0.11 ± 0.14 logarithm minimum angle resolution (logMAR). FFAmp decreased by 3.01 5.9 µV global deteriorating 18.7% annually. percentage change (RE [r = 0.553], LE 0.531]), 30 0.615], 0.529]) strongly correlated (P < 0.00001). p50 (15 30 degrees) analyzed 34 showed reduction 23% 23.4%, respectively. 30 degrees (r 0.397) EZ layer 0.05). calculated 45 the shortening thinning rate 4.3% 4.4% annually, length 0.34] 0.466; P Conclusions We quantified utilizing measures residual function. These parameters when measurable. objective may assist monitoring disease progression. Translational Relevance Residual provides an estimate function, which aids counselling regarding prognosis.

参考文章(50)
Ann H Milam, Zong-Yi Li, Robert N Fariss, Histopathology of the human retina in retinitis pigmentosa Progress in Retinal and Eye Research. ,vol. 17, pp. 175- 205 ,(1998) , 10.1016/S1350-9462(97)00012-8
E L Berson, Retinitis pigmentosa. The Friedenwald Lecture. Investigative Ophthalmology & Visual Science. ,vol. 34, pp. 1659- 1676 ,(1993)
Birgit Sander, Hajer Ahmad Al-Abiji, Mads Kofod, Thomas Martini Jørgensen, Do different spectral domain OCT hardwares measure the same? Comparison of retinal thickness using third-party software. Graefes Archive for Clinical and Experimental Ophthalmology. ,vol. 253, pp. 1915- 1921 ,(2015) , 10.1007/S00417-015-3075-2
Carol Weigel-DiFranco, Bernard Rosner, Michael A. Sandberg, Thaddeus P. Dryja, Eliot L. Berson, Disease progression in patients with dominant retinitis pigmentosa and rhodopsin mutations. Investigative Ophthalmology & Visual Science. ,vol. 43, pp. 3027- 3036 ,(2002)
Jane L Anderson, Gary E Fish, David G Birch, Yearly rates of rod and cone functional loss in retinitis pigmentosa and cone-rod dystrophy. Ophthalmology. ,vol. 106, pp. 258- 268 ,(1999) , 10.1016/S0161-6420(99)90064-7
Rouel S. Roque, Christopher J. Imperial, Ruth B. Caldwell, Microglial Cells Invade the Outer Retina as Photoreceptors Degenerate in Royal College of Surgeons Rats Investigative Ophthalmology & Visual Science. ,vol. 37, pp. 196- 203 ,(1996)
Ebrahim Jafarzadehpur, Hassan Hashemi, Homa Hassan-Karimi, Bahram Blouri, Arash Zare Sadeghi, Ali Mirzajani, Frequency Domain Electroretinography in Retinitis Pigmentosa versus Normal Eyes Journal of ophthalmic and vision research. ,vol. 7, pp. 34- 38 ,(2012)
D Hamasaki, The a-Wave Latency in Control Subjects and Patients with Retinal Diseases Japanese Journal of Ophthalmology. ,vol. 46, pp. 433- 442 ,(2002) , 10.1016/S0021-5155(02)00504-X
Samuel G. Jacobson, Alejandro J. Roman, Tomas S. Aleman, Alexander Sumaroka, Waldo Herrera, Elizabeth A. M. Windsor, Lori A. Atkinson, Sharon B. Schwartz, Janet D. Steinberg, Artur V. Cideciyan, Normal central retinal function and structure preserved in retinitis pigmentosa. Investigative Ophthalmology & Visual Science. ,vol. 51, pp. 1079- 1085 ,(2010) , 10.1167/IOVS.09-4372