Bilateral Symmetry of Visual Function Loss in Cone-Rod Dystrophies

被引:12
作者
Galli-Resta, Lucia [1 ]
Falsini, Benedetto [2 ]
Rossi, Giuseppe [3 ]
Piccardi, Marco [2 ]
Ziccardi, Lucia [4 ]
Fadda, Antonello [5 ]
Minnella, Angelo [2 ]
Marangoni, Dario [2 ]
Placidi, Giorgio [2 ]
Campagna, Francesca [2 ]
Abed, Edoardo [2 ]
Bertelli, Matteo [6 ]
Zuntinif, Monia [6 ]
Resta, Giovanni [7 ]
机构
[1] CNR, Ist Neurosci, Pisa, Italy
[2] Catholic Univ, Policlin Gemelli, Inst Ophthalmol, Rome, Italy
[3] CNR, Ist Fisiol Clin, Pisa, Italy
[4] IRCCS, Bietti Fdn, Rome, Italy
[5] Ist Super Sanita, Tecnol & Salute, Rome, Italy
[6] MAGI Lab Med Genet, Rovereto, Italy
[7] CNR, Ist Informat & Telemat, Pisa, Italy
关键词
cone dystrophy; interocular; visual acuity; visual function; electroretinography; PIGMENT OPTICAL-DENSITY; STARGARDT DISEASE; GENE-THERAPY; RETINITIS-PIGMENTOSA; PROGRESSIVE CONE; DEGENERATIONS; POPULATION; DISORDERS;
D O I
10.1167/iovs.15-18313
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
PURPOSE. To investigate bilateral symmetry of visual impairment in cone-rod dystrophy (CRD) patients and understand the feasibility of clinical trial designs treating one eye and using the untreated eye as an internal control. METHODS. This was a retrospective study of visual function loss measures in 436 CRD patients followed at the Ophthalmology Department of the Catholic University in Rome. Clinical measures considered were best-corrected visual acuity, focal macular cone electroretinogram (fERG), and Ganzfeld cone-mediated and rod-mediated electroretinograms. Interocular agreement in each of these clinical indexes was assessed by t- and Wilcoxon tests for paired samples, structural (Deming) regression analysis, and intraclass correlation. Baseline and follow-up measures were analyzed. A separate analysis was performed on the subset of 61 CRD patients carrying likely disease-causing mutations in the ABCA4 gene. RESULTS. Statistical tests show a very high degree of bilateral symmetry in the extent and progression of visual impairment in the fellow eyes of CRD patients. CONCLUSIONS. These data contribute to a better understanding of CRDs and support the feasibility of clinical trial designs involving unilateral eye treatment with the use of fellow eye as internal control.
引用
收藏
页码:3759 / 3768
页数:10
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