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Retinitis Pigmentosa (Pigmentary Retinopathy)

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Hereditary, progressive degeneration of the neuroepithelium of the retina characterized by night blindness and progressive contraction of the visual field.

Also Known As:

Pigmentary Retinopathy; Retinopathy, Pigmentary; Tapetoretinal Degeneration; Cone-Rod Degenerations; Cone-Rod Dystrophy; Rod Cone Dystrophies; Cone Rod Degenerations; Cone Rod Dystrophy; Cone-Rod Degeneration; Cone-Rod Dystrophies; Degeneration, Cone-Rod; Degeneration, Tapetoretinal; Degenerations, Cone-Rod; Degenerations, Tapetoretinal; Dystrophies, Cone-Rod; Dystrophies, Rod Cone; Dystrophies, Rod-Cone; Dystrophy, Cone-Rod; Dystrophy, Rod Cone; Dystrophy, Rod-Cone; Pigmentary Retinopathies; Retinopathies, Pigmentary; Rod Cone Dystrophy; Rod-Cone Dystrophies; Tapetoretinal Degenerations; Rod-Cone Dystrophy

Networked: 2585 relevant articles (54 outcomes, 210 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Macular Edema
2.	Macular Degeneration (Age-Related Maculopathy)
3.	Eye Diseases (Eye Disease)
4.	Retinal Dystrophies
5.	Retinal Degeneration

Experts

1.	Berson, Eliot L: 34 articles (10/2014 - 03/2002)
2.	Swaroop, Anand: 25 articles (07/2015 - 01/2003)
3.	Jacobson, Samuel G: 21 articles (10/2015 - 04/2002)
4.	Aguirre, Gustavo D: 20 articles (10/2015 - 04/2002)
5.	Tsang, Stephen H: 20 articles (09/2015 - 03/2007)
6.	Sahel, José-Alain: 20 articles (05/2015 - 05/2004)
7.	Bhattacharya, Shomi S: 20 articles (10/2014 - 01/2002)
8.	Cheetham, Michael E: 19 articles (01/2016 - 07/2002)
9.	Cremers, Frans P M: 19 articles (11/2015 - 12/2005)
10.	den Hollander, Anneke I: 19 articles (11/2015 - 03/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Retinitis Pigmentosa:

1.	Retinaldehyde (Retinal)IBA 01/01/2011 - "Recently, unoprostone has been tested in clinical studies as a therapeutic agent for retinitis pigmentosa (RP) and studies have demonstrated an improvement in retinal sensitivity and in the protection of central retinal sensitivity with its use. " 11/01/2012 - "These results demonstrate for the first time that gene therapy effectively restores long-term retinal function and vision in a large animal model of autosomal recessive rod-cone dystrophy, and provide great promise for human treatment." 04/01/2009 - "Improved retinal function in a mouse model of dominant retinitis pigmentosa following AAV-delivered gene therapy." 10/01/2015 - "This study investigated whether voluntary running wheel exercise can be neuroprotective in an inheritable model of the retinal degenerative disease retinitis pigmentosa (RP), rd10 mice. " 01/01/2015 - "In the present study we have isolated retinal cells from rats with inherited retinitis pigmentosa (RP) during the post-natal stage of photoreceptor degenerative. "
2.	dorzolamide (Trusopt)FDA LinkGeneric 09/01/2013 - "Therapeutic effect of prolonged treatment with topical dorzolamide for cystoid macular oedema in patients with retinitis pigmentosa." 06/01/2007 - "Continued use of dorzolamide for the treatment of cystoid macular oedema in patients with retinitis pigmentosa." 09/01/2010 - "To determine the efficacy of sustained topical therapy with dorzolamide hydrochloride, 2%, on visual acuity and cystic macular lesions in patients with retinitis pigmentosa and Usher syndrome. " 09/01/2010 - "Efficacy of sustained topical dorzolamide therapy for cystic macular lesions in patients with retinitis pigmentosa and usher syndrome." 01/01/2014 - "To assess the efficacy of topical dorzolamide for treating cystoid macular edema in patients with retinitis pigmentosa and minimize the secondary effects of maintenance therapy in patients with retinitis pigmentosa (RP) who present with chronic microcystic macular edema. "
3.	Rhodopsin (Visual Purple)IBA 01/01/2014 - "Following a previous study that demonstrated a correlation between rhodopsin stability and the severity of retinitis pigmentosa (RP), we investigated whether predictions of severity can be improved with a regional analysis of this correlation. " 02/05/2010 - "Molecular mechanisms of rhodopsin retinitis pigmentosa and the efficacy of pharmacological rescue." 01/01/2014 - "This study was to analyze the spectrum and frequency of rhodopsin gene (RHO) mutations in Chinese patients with retinitis pigmentosa (RP). " 01/26/2012 - "Understanding rhodopsin mutations linked to the retinitis pigmentosa disease: a QM/MM and DFT/MRCI study." 03/01/2011 - "[Structural development study of a novel pharmacological chaperone for folding-defective rhodopsin mutants responsible for retinitis pigmentosa]."
4.	Acetazolamide (Diamox)FDA LinkGeneric 05/01/1993 - "The therapeutic effect of long-term treatment with acetazolamide (from 3 weeks to 16 months) of seven patients with retinitis pigmentosa was studied. " 05/01/1993 - "Long-term treatment of retinitis pigmentosa with acetazolamide. " 01/01/1991 - "In the patients with retinitis pigmentosa, the hyperosmolarity response and light rise were decreased, while the acetazolamide response was normal. " 09/01/1990 - "Long-term effect of acetazolamide in a patient with retinitis pigmentosa." 01/01/2001 - "To investigate whether acetazolamide, already found to be helpful in decreasing cystoid macular edema in patients with retinitis pigmentosa, was also effective in the treatment of diabetic macular edema in nonproliferative retinopathy. "
5.	Ciliary Neurotrophic Factor (CNTF)IBA 10/01/2002 - "The objective of the present study was to evaluate the therapeutic efficacy of ciliary neurotrophic factor (CNTF) delivered through encapsulated cells directly into the vitreous of the eye in an rcd1 canine model of retinitis pigmentosa. " 11/01/2001 - "The present study aimed to determine whether intravitreal administration of an adeno-associated virus (AAV) carrying ciliary neurotrophic factor (CNTF) can achieve long-term morphological and physiological rescue of photoreceptors in animal models of retinitis pigmentosa, and whether injection of this virus after degeneration begins is effective in protecting the remaining photoreceptors. " 10/01/2014 - "Thus far, CNTF has failed to demonstrate rod protection in trials for human retinitis pigmentosa. " 08/01/2013 - "Randomized trial of ciliary neurotrophic factor delivered by encapsulated cell intraocular implants for retinitis pigmentosa." 04/01/2007 - "This study was conducted to test whether CNTF prevents photoreceptor cell loss in XLPRA2, an early onset canine model of X-linked retinitis pigmentosa caused by a frameshift mutation in RPGR exon ORF15. "
6.	Vitamin A (Retinol)FDA LinkGeneric 01/01/2001 - "Oral vitamin A therapy has proven to be effective in the treatment of the common forms of retinitis pigmentosa. " 09/01/2004 - "In patients assigned to receive 15 000 IU/d of vitamin A, this randomized trial showed that 1200 mg/d of docosahexaenoic acid supplementation over a 4-year interval did not, on average, slow the course of disease in patients with retinitis pigmentosa." 01/01/1982 - "Approaches to human retinitis pigmentosa through studies of vitamin A metabolism in the RCS (dystrophic) rat." 02/01/2013 - "ω-3 Intake in patients with retinitis pigmentosa receiving vitamin A-Reply." 02/01/2013 - "ω-3 Intake in patients with retinitis pigmentosa receiving vitamin A."
7.	Valproic Acid (Valproate, Semisodium)FDA LinkGeneric 09/01/2014 - "To evaluate the efficacy of valproic acid (VPA) on visual function in patients with retinitis pigmentosa (RP). " 09/01/2014 - "Efficacy of oral valproic acid in patients with retinitis pigmentosa." 12/01/2012 - "The aim of this study was to evaluate the effect of short-term oral valproic acid (VPA) on the vision and visual fields of patients with retinitis pigmentosa (RP). " 07/01/2013 - "Long-term follow-up for efficacy and safety of treatment of retinitis pigmentosa with valproic acid." 12/01/2012 - "Effect of short-term oral valproic Acid on vision and visual field in retinitis pigmentosa."
8.	Nerve Growth Factors (Neurotrophins)IBA 03/01/1997 - "Because apoptotic cell death has been shown to be the common terminal fate of photoreceptors in the rd mouse and many other animal models of retinitis pigmentosa, these results suggest the possibility that administration of neurotrophic factors may prove beneficial in reducing photoreceptor loss in multiple forms of retinitis pigmentosa." 09/01/2009 - "[Progress in treatment of retinitis pigmentosa by neurotrophic factors]." 03/07/2006 - "Neurotrophic factors are agents with a promising ability to retard progression of neurodegenerative diseases and are effective in slowing photoreceptor degeneration in animal models of retinitis pigmentosa. " 09/01/2012 - "Glial cell line-derived neurotrophic factor (GDNF) is one of the candidate molecules among neurotrophic factors proposed for a potential treatment of retinitis pigmentosa (RP). " 05/30/2006 - "The aim of this study was to investigate the effect of electroacupuncture (EA) on the progression of retinal degeneration in rats affected by inherited retinitis pigmentosa (IRP) and to correlate this event with the retinal expression of neurotrophins. "
9.	Vitamin E FDA LinkGeneric 03/01/2006 - "Finally, the methods are illustrated with two data sets, (i) an ophthalmologic data set involving a comparison of electroretinogram (ERG) data in retinitis pigmentosa (RP) patients before and after undergoing an experimental surgical procedure, and (ii) a nutritional data set based on a randomized prospective study of nutritional supplements in RP patients where vitamin E intake outside of study capsules is compared before and after randomization to monitor compliance with nutritional protocols." 03/01/2014 - "In very high-risk premature retinopathy and retinitis pigmentosa, antioxidant supplements may be beneficial but those with excess Vitamin E should be avoided. " 01/01/1994 - "Our result could explain the observation that vitamin E supplements have an adverse effect on light-damaged retina and on the course of retinitis pigmentosa. " 01/01/1957 - "[Vitamin E & pigmentary retinopathy; determination of total tocopherol in blood]." 01/01/2001 - "Vitamin E therapy for Friedreich-like ataxia associated with retinitis pigmentosa has been shown to be effective in the short term. "
10.	DNA (Deoxyribonucleic Acid)IBA 04/01/2010 - "I. Gene delivery to mitotic and postmitotic photoreceptors via compacted DNA nanoparticles results in improved phenotype in a mouse model of retinitis pigmentosa." 04/01/2010 - "Gene delivery to mitotic and postmitotic photoreceptors via compacted DNA nanoparticles results in improved phenotype in a mouse model of retinitis pigmentosa." 03/01/2011 - "The detection of known and novel retinitis pigmentosa mutations in this study establishes high-throughput DNA sequencing with DNA pooling as an effective diagnostic tool for heterogeneous genetic diseases." 04/01/2010 - "The purpose of the present study was to test the therapeutic efficiency and safety of compacted-DNA nanoparticle-mediated gene delivery into the subretinal space of a juvenile mouse model of retinitis pigmentosa. " 11/01/2013 - "DNA samples from 797 unrelated patients with LCA and 211 with the various types of retinitis pigmentosa (RP) were screened by Sanger sequence analysis of all LCA5 exons and intron/exon junctions. "

Therapies and Procedures

1.	Transplantation (Transplant Recipients) 01/01/2012 - "This study examines the efficacy of iPS transplantation in restoring functional vision in Rpe65(rd12)/Rpe65(rd12) mice, a clinically relevant model of retinitis pigmentosa (RP). " 08/01/2004 - "To report the subjective and objective improvement in vision in a patient with autosomal dominant retinitis pigmentosa after transplantation of a sheet of fetal neural retina together with its retinal pigment epithelium. " 05/01/1999 - "The transplantation of human fetal neuroretinal cells in advanced retinitis pigmentosa patients: results of a long-term safety study." 03/25/2011 - "Further investigation of intraretinal transplantation of olfactory ensheathing cells may suggest a possible future route for protection of the retina in retinitis pigmentosa." 10/01/2010 - "Transplantation of reprogrammed embryonic stem cells improves visual function in a mouse model for Retinitis Pigmentosa: Transplantation 2010 April 27;89 (8): 911-919."
2.	Visual Prosthesis 06/01/2015 - "The prospective efficacy of a future peripheral retinal prosthesis complementing residual vision to raise mobility performance in non-end stage retinitis pigmentosa (RP) was evaluated using simulated prosthetic vision (SPV). " 06/01/2014 - "To study the anatomic and functional outcomes of Argus II Retinal Prosthesis System implantation in patients with retinitis pigmentosa. " 01/01/2014 - "The objective of this study was to assess the cost-effectiveness of the Argus® II Retinal Prosthesis System (Argus II) in Retinitis Pigmentosa (RP) patients. " 07/01/2013 - "Since 2000, several groups have initiated chronic studies, implanting electronic retinal prostheses into the blind eyes of patients with retinitis pigmentosa to produce formed vision. " 07/01/2012 - "We undertook a questionnaire study to explore the retinitis pigmentosa (RP) patients' attitudes and the main reasons for or against participation in retinal prosthesis trials. "
3.	Vitrectomy 01/01/2001 - "To describe a patient with bilateral retinitis pigmentosa and visually disabling vitreous opacities, who benefited from vitrectomy in both eyes. " 03/01/2011 - "To determine the prevalence of macular abnormalities detected by optical coherence tomography (OCT) in patients with retinitis pigmentosa (RP), and to report the results of pars plana vitrectomy (PPV) in five patients with RP. " 01/01/2001 - "Vitrectomy for large vitreous opacity in retinitis pigmentosa." 01/01/2014 - "Microincision vitrectomy surgery in vitreomacular traction syndrome of retinitis pigmentosa patients." 05/01/2011 - "A male patient with retinitis pigmentosa (RP) accompanied by repeated vitreous hemorrhage in both eyes underwent unilateral vitrectomy. "
4.	Transplants (Transplant) 01/01/2012 - "Long-term safety and efficacy of human-induced pluripotent stem cell (iPS) grafts in a preclinical model of retinitis pigmentosa." 06/01/2007 - "Long-term neuroretinal full-thickness transplants in a large animal model of severe retinitis pigmentosa." 08/01/2004 - "Vision change after sheet transplant of fetal retina with retinal pigment epithelium to a patient with retinitis pigmentosa." 06/01/2007 - "The purpose of this study was to explore neuroretinal transplantation in a large animal model of severe retinitis pigmentosa and to establish graft development, long-term survival, graft-host integration, and effects on the host retina. " 09/01/2000 - "Eight patients with bare light perception (LP) vision due to retinitis pigmentosa (RP) and one patient with bare LP vision due to advanced neovascular age-related macular degeneration (AMD) received subretinal transplants of human fetal retinal microaggregate suspensions without postoperative systemic immunosuppression. "
5.	Electrodes (Electrode) 01/01/2009 - "The purpose of this study was to evaluate the efficacy and safety of artificial vision by using a direct optic nerve electrode (AV-DONE) in a blind patient with retinitis pigmentosa (RP). " 06/01/2012 - "Previously, we reported on the responses of RGCs in the P23H rat (a model of retinitis pigmentosa) and the Sprague-Dawley (SD) rat to stimulation with a 400-μm diameter electrode (Jensen and Rizzo, 2011). " 01/01/2011 - "Since 2002, six blind patients have undergone implantation of an epiretinal 4 × 4 electrode array designed to directly stimulate the remaining cells of the retina after severe photoreceptor degeneration due to retinitis pigmentosa. " 01/01/2009 - "Artificial vision by direct optic nerve electrode (AV-DONE) implantation in a blind patient with retinitis pigmentosa." 04/01/2003 - "Pulses of low intensity current, delivered through a cuff electrode chronically implanted around the optic nerve of a blind retinitis pigmentosa patient generate visual sensations. "