Gene Ther. [49][50] Therefore, postmenopausal women and men over the age of 49 who are taking vitamin A should consult with their primary care physician regarding their bone health. There is often a high degree of symmetry in the fundus findings between the two eyes. A worm can be found with careful examination of the fundus. These include: Dilated Eye Exam During a dilated eye exam, you are given special eye drops to dilate your pupils, allowing your ophthalmologist to clearly see the retina at the back of your eye. Retinitis pigmentosa is a clinically and genetically heterogeneous group of hereditary disorders in which there is progressive loss of photoreceptor and pigment epithelial function. The later may have a 'bone corpuscle' appearance with a perivascular distribution. Finally, vitamin A should not be given to patients on chronic doxycycline because the combination can lead to increased intracranial pressure. It has been speculated that vitamin A rescues remaining cones, thereby explaining how one supplement may help a group of patients with different rod-specific gene defects. Feskanich D, Singh V, Willett WC, Colditz GA: Vitamin A intake and hip fractures among postmenopausal women. [2] The b/a amplitude ratio is typically decreased, when it is below 1.0, a negative ERG pattern is found. Retinitis pigmentosa (RP) is a genetically heterogeneous disease with 89 causative genes identified to date. 2000;25(3):306-310. The ERG, in conjunction with the visual field exam, will usually make the diagnosis. Arch Ophthalmol. There are two promising treatments in development for this treatment. Cox SN, Hay E, Bird AC: Treatment of chronic macular edema with acetazolamide. In: Newsome DA, ed. Am J Ophthalmol. BMC Ophthalmol. [7], Patients with RP characteristically develop night blindness and difficulty with mid-peripheral visual field in adolescence. Ali RR, Sarra GM, Stephens C, et al. Sector retinitis pigmentosa is an atypical form of RP, which is characterized by regionalized areas of bone spicule . b) Bone specular pigmentation starting in the foveal center. Carbonic anhydrase inhibitors such as acetazolamide have been used at a daily dosage of 500 mg or less.19 Close monitoring is needed, as there is a risk of rebound intraretinal fluid accumulation with continued use.20 Similarly, topical dorzolamide has been successful in treating CME, although rebound effects have also been observed in some cases.19,21 Intravitreal or sub-Tenon injections of triamcinolone acetonide have been tried, but the effects have generally not been sustained.19, AIR. Br J Ophthalmol. 2008;146(3):417-426. If the patient develops a cataract, it is generally advisable to defer surgical removal until the patient can no longer read with the better eye. Design: This is a single center, observational, cross-sectional repeatability study of patients with retinitis pigmentosa. Leber congenital amaurosis is a form of retinitis pigmentosa that affects infants. Is a 159 gene panel that includes assessment of non-coding variants. 2. Retinitis pigmentosa (RP) comprises a complex group of inherited dystrophies characterized by progressive degeneration and dysfunction of the retina, primarily affecting photoreceptor and pigment epithelial function [ 1 ]. This strip encircles the eyeball and is surgically sewn onto the sclera. Profile of clinical findings. Wright AF, Jay B, eds. Clipboard, Search History, and several other advanced features are temporarily unavailable. These patients constitute a recognizable subgroup of RP, independent of the mode of inheritance, which is characterized by delimited disease, mild functional symptoms, and a good visual prognosis. Central vision typically declines in the advanced stages of retinitis . The characteristic pigmentary changes occur in the mid-peripheral fundus, which is predominantly populated by rods. The ERG is a test used worldwide to assess the status of the retina in eye diseases in human patients and in laboratory animals used as models of retinal disease. A clinical, psychophysical, and electroretinographic survey of patients with autosomal dominant retinitis pigmentosa. e) Secondary optic atrophy with greyish dirty disc appearance. Infectious causes including syphilis, rubella, and diffuse unilateral subacute neuroretinitis (DUSN) can cause similar pigmentary retinopathy seen in patients with RP. 2010;128(4):403-411. Epub 2012 Jun 5. 18 Zhang Q. Asia Pac J Ophthalmol (Phila). doi: 10.1001/archopht.1979.01020020042009. 1983;24(4):458-469. 1983 Jul;67(7):419-30. doi: 10.1136/bjo.67.7.419. Correspondence Arch Ophthalmol. Recently, testing for genetic defects is being done to clarify the loss in more detail and to find a treatment. Retinitis pigmentosa (RP) is a group of inherited progressive retinal dystrophies that is present with progressive vision loss, night blindness, visual field reduction, and retinal pigmentation of the fundus. connectcenter@aph.org. eCollection 2016. In: Albert D, Miller J, Azar D, Blodi B, eds. What to Tell Your Employer When Youre Losing Your Sight, Working with People New to Visual Impairment, Getting Started 2020: A Guide for People New to Vision Loss, http://www.medicinenet.com/retinitis_pigmentosa/article.htm, Download the Getting Started Guide in English (accessible pdf), Descargue la Gua de inicio en espaol (pdf accesible), Register your agency for the Directory of Services, Decreased vision at night or in low light. Low vision services are designed to benefit those whose ability to function is compromised by visual impairment. Koushik Tripathy, MD (AIIMS), FRCS (Glasgow), Dr John Davis Akkara (MBBS, MS, FAEH, FMRF), Carver Nonprofit Genetic Testing Laboratory at the University of Iowa, diffuse unilateral subacute neuroretinitis (DUSN), https://www.aao.org/eye-health/diseases/retinitis-pigmentosa-list, https://eyewiki.org/w/index.php?title=Retinitis_Pigmentosa&oldid=81059. It is important to note that anecdotal treatment with a subjective improvement of the visual function should be interpreted with caution due to fluctuation in visual acuity and visual fields in this disease. 615720. Over 80 genes have been attributed to RP resulting in significant clinical heterogeneity. ERG is the most important basis for differential diagnosis of . Invest Ophthalmol Vis Sci. Replacement of the gene in younger patients (versus adults) was shown to improve functional vision based on multi-luminance mobility testing, which scored subjects' ability to navigate a standardized maze under different levels of illumination. Different treatments are utilized to treat retinitis pigmentosa. E. 20-Retinitis pigmentosa is characterized by: a) Color blindness. Retinitis Pigmentosa. Prevalence of non syndromic RP is approximately 1/4,000. Clinical Trials for Retinitis Pigmentosa treatments are still ongoing, and one of the latest treatments is the N-acetylcysteine. Other indicators of retinitis pigmentosa are your family history (especially the possibility of retinitis pigmentosa appearing in other family members) and expressed visual concerns or complaints, such as not being able to see well at night or in low light conditions. It may not be noticed by the person with retinitis pigmentosa, but may be apparent to others. 1988;106(3):369-375. Methods . NOTE: OMIM is intended for use primarily by physicians and other professionals concerned with genetic disorders, by genetics researchers, and by advanced students in science and medicine. da Cruz L, Coley BF, Dorn J, Merlini F, Filley E, Christopher P, Chen FK, Wuyyuru V, Sahel J, Stanga P, Humayun M, Greenberg RJ, Dagnelie G; Argus II Study Group. Periodic follow-up ERG examinations are necessary to follow and track the progression of your retinitis pigmentosa. Br J Ophthalmol. Dark adaptometry (DA): Visual threshold is the minimum intensity of light that will stimulate the rods or cones to elicit a subjective response. 11 Bernstein PS et al. 2015;2015:737053. doi:10.1155/2015/737053. Progression of RP was evaluated with MD slope and the average sensitivity of the central 2 (CENT4). If you're a patient or visitor in one of our hospitals or clinics, you're required to wear a mask indoors. A series of tests are available to confirm a diagnosis of RP. Chow AY, Pardue MT, Perlman JI, et al. Although light deprivation has not been shown to be of benefit in altering the course of retinal degeneration,[28] it is generally advisable for patients to use ultraviolet and short-wavelength (blue) blocking sunglasses for outdoor activities. Philadelphia, PA: Elsevier; 2008:Ch. Most critical diagnostic test for retinitis pigmentosa. Hereditary pigmentary degenerations of the retina. Rubella is usually characterized by normal retinal vessels and normal visual function as documented by visual acuity, visual field, and ERG. Retinitis pigmentosa (RP) is the name given to a group of inherited eye diseases that affect the retina (the light-sensitive part of the eye). See Page 1. Arch Ophthalmol. 2014 Dec 31. Full-Field Electroretinogram (ERG): ERG measures the electrical potential generated by rods and cones after a light stimulus and is essential in the diagnosis of RP. Bookshelf Apart from accounting for 3% of autosomal recessive RP, ABCA4 gene mutations also cause cone dystrophies, cone-rod dystrophies, and Stargardt disease.5 A study by Radu et al. 36 The sponsor is currently recruiting patients for its randomized, double-masked Phase II RESTORE trial to assess the efficacy and safety profiles of the therapy with a timeframe of one year. Retinitis pigmentosa, Usher's syndrome, and other pigmentary retinopathies. Curr Eye Res. Treatment consists of restricting food items that contain phytanic acid (including animal fats, milk products, and green leafy vegetables containing phytol) while maintaining body weight. The ERG will also determine if there is any involvement of the central retina and visual field. [5] In the rat, retinal degeneration caused by the failure of retinal pigment epithelium to phagocytose the shed rod outer segment discs results in the accumulation of rod outer segment debris. Clinical ophthalmology. 2008;19(10):979-990. Other genetic disorders that cause retinal degeneration include gyrate atrophy, choroideremia, cone-rod dystrophy, cone dystrophy, and Leber congenital amaurosis. X-linked is the most severe form with appreciable impairment of central visual acuity to 20/200 or less by the fifth decade of life. This prospective, sequential, open-label phase-3 clinical study was conducted at Ankara University Faculty of Medicine, Department of Ophthalmology . Patients with syphilis will have positive anti-treponemal antibody tests such as fluorescent treponemal antibody-absorbed (FTA-ABS) or microhemagglutination assay for Treponema pallidum (MHA-Tp) and should improve with proper treatment. 2016;50:34-66. ), With the advent of genetic studies, many different treatment methods for RP are currently being explored. Other measures that patients with RP may find helpful in coping with their vision loss include participation in vision rehabilitation clinics and the use of visual aids such as magnifiers and night vision devices.3. 1980;20(12):1179-1184. 2006; 141(5):850-858. N-acetylcysteine or NAC comes from the amino acid L-cysteine which is a building block of protein. 2, 22 In contrast, our patient presented with advanced retinitis pigmentosa and severe visual decline. In: Duane TD, Jaeger EA, eds. Timing of onset can vary among pedigrees. Arch Ophthalmol. In the second study, patients who were given vitamin A palmitate 15,000 IU/day were randomized to either DHA capsules (1200 mg/day) or control fatty acid capsules. MeSH 2012;18:1478-83. 2004;137(4):704-718. Retinal prostheses. 2008;358(21):2231-2239. Lang GE, Maumenee IH. Nat Genet. Yuzbasioglu E, Artunay O, Rasier R, et al. [8], Patients typically present with night vision problems (unable to see in the dark or slow to adjusting to dark), progressive peripheral vision restriction, and tunnel vision at the later stage of the disease. Would you like email updates of new search results? Patients with the same gene defect can have variable severity of disease at a given age. Arch Ophthalmol. Marmor MF. 3. Graefes Arch Clin Exp Opthalmol. 14. Affected status was determined by extinguished responses on electroretinogram (ERG) and/or symptoms characteristic of RP, including nyctolopia and peripheral visual field loss. examined the effects of different doses of encapsulated CNTF intraocular implants in patients with early and late RP.14 There was no significant difference in the best-corrected VA of patients in the high-dose versus sham and low-dose versus sham groups in either study.14 Both studies also found that patients with high-dose implants had decreased visual field sensitivity compared to those with sham implants at 12 months, although this difference became statistically insignificant six months after removal of the implant.14, Other agents. electrophysiologic functional tests are also essential. Audiology consults should be considered for patients with a possible or known diagnosis of Usher syndrome. [6] Genes that cause RP can be categorized into those that affect the phototransduction cascade, the retinoid cycle, photoreceptor structure, or other biological function of photoreceptor and retinal pigment epithelium. Federal government websites often end in .gov or .mil. A low vision examination may be useful to help optimize the use of the remaining visual function. There is an FDA approved Humanitarian Device, called the ARGUS II implant, which may help patients with end-stage RP. Ophthalmology. Michaelsson K, Lithell H, Vessby B, Melhus H: Serum retinol levels and the risk of fracture. Home modification and education on low vision aids help patients maximize their remaining functional vision. [21][22] In the 2nd and 3rd study, conclusions were drawn based on secondary outcomes and subgroup analyses, rather than the primary outcome. N Engl J Med. 7 Hoffman DR et al. Berson EL, Rosner B, Sandberg MA, et al: Vitamin A supplementation for retinitis pigmentosa. Arch Ophthalmol. Genetic testing can be helpful in confirming the diagnosis. Br J Ophthalmol 2013 May;97(5):632-6. Autosomal dominant RP has the best prognosis, with the majority of patients under 30 years having a visual acuity of 20/30 or better. ERG measures the electrical potential generated by rods and cones after a light stimulus and is essential in the diagnosis of retinitis pigmentosa. Vitamin E may lead to an adverse effect on the course of RP by inhibiting the absorption or transport of vitamin A. DHA is thought to facilitate the release of vitamin A from its carrier protein (interphotoreceptor retinoid-binding protein) in the subretinal space. Symptoms, including loss of vision or visual sharpness, usually begin in childhood or adolescence. This article focuses on typical RP with a brief mention of rare but treatable types of RP. The retinitis pigmentosa 2 polypeptide (RP2) functions as a GTPase-activating protein (GAP) for ARL3 (Arf-like protein 3), a small GTPase. Examination of electroretinographic (ERG) reports from 70 consecutive patients with retinitis pigmentosa (RP) showed that photopic and scotopic B-wave amplitu . 13 Berson EL et al. In RP, the photoreceptors progressively lose function, and side or peripheral vision and night vision slowly worsens over time. In patients with another form of RP, Leber's variant, gene therapy for RPE65 is available and FDA-approved. In older adults, long-term vitamin A supplementation has been associated with a decrease in bone density and up to a 1% increased risk of hip fractures. Visual loss. Retinitis pigmentosa (RP) comprises a large group of inherited vision disorders that cause progressive degeneration of the retina (the so-called inherited retinal diseases, or IRDs), the light sensitive membrane that coats the inside of the eyes. Retinitis pigmentosa (RP) refers to a heterogeneous group of inherited disorders that are characterized by loss of retinal cell function, preferentially in the peripheral retina. 2005;26(2):91-100. 2000 Feb;107(2):386-96. doi: 10.1016/s0161-6420(99)00045-7. A similar pattern appears in congenital syphilis, which is unrelated and must be ruled out. DH, Chen A, Arevalo JF, Birch DG, Branham K, Coley B, Dagnelie G, Juan ED; Support groups may also be beneficial to patients in managing psychosocial difficulties. It is rare for patients to lose all vision in both eyes. [26] The long-term efficacy of topical dorzolamide in improving the macular cystic lesions in patients with RP and Usher syndrome has been demonstrated in a retrospective series with a mean follow-up of 39 months.[27]. Massof RW, Finkelstein D: Supplemental vitamin A retards loss of ERG amplitude in retinitis pigmentosa. Retinitis pigmentosa (RP) comprises a group of inherited retinal dystrophies that primarily affects the normal function of rod photoreceptor cells in the retina. Discov Med. Figure 4: Genes and their relative contribution to retinitis pigmentosa Causal genes and their contributions to (A) autosomal-recessive disease (ARRP), including Usher's and Bardet-Biedl (BBS) syndromes, (B) autosomal-dominant disease, and (C) X-linked disease. Philadelphia, PA: Elsevier; 2008:Ch. Photopic electroretinogram implicit time in retinitis pigmentosa. Retinitis pigmentosa (opthalmology) Ma Wady 4.1k views 10 slides Cone and Rod Dystrophy Dr Samarth Mishra 3.1k views 81 slides hereditary macular and choroidal dystrophies Priyanka Choudhary 3k views 34 slides Retinitis pigmentosa kamalinineha6 2.5k views 59 slides Nw2016 retinitis pigmentosa Nawat Watanachai 3.3k views 18 slides If medical treatment is initiated, more frequent visits and laboratory blood work may be indicated. Initial visual problems appear during adolescence and manifest in the form of reduced nighttime vision and diminished peripheral vision. Certain conditions including complete congenital stationary night blindness, melanoma-associated retinopathy, and juvenile X-linked retinoschisis produce a characteristic abnormality of this response that has been termed "electronegative." Specifically, the a-wave has a normal (or nearly normal) amplitude, whereas the b-wave is markedly attenuated. Hum Mol Genet. The video unit converts the video images into electrical impulses which are transmitted to the receiver. Berson EL, Rosner B, Sandberg MA, et al. SLC7A14. J Ophthalmol. Beyond Sector Retinitis Pigmentosa: Expanding the Phenotype and Natural History of the Rhodopsin Gene Codon 106 Mutation (Gly-to-Arg) in Autosomal Dominant Retinitis Pigmentosa. 2004; 122(9):1297-1305. Macular pigment optical density correlates linearly with the concentration of macular pigments such as lutein and zeaxanthin and has been found to be lower in eyes with retinal diseases such as age-related macular degeneration and Stargardt disease but not in those with RP.11 Nevertheless, given the protective role that lutein plays in the retina, supplementation has been studied as a form of treatment in RP. Rescue of photoreceptor function by AAV mediated gene transfer in a mouse model of inherited retinal degeneration. Patients often have a good central vision from a small central island (tunnel vision) until their 50s or 60s. There are many genes associated with RP for which a patient can undergo genetic testing. Two randomized controlled trials by Hoffman et al. Retinitis pigmentosa. Philadelphia, PA: JB Lippincott; 1988:221-252. Massof RW, Finkelstein D. Supplemental vitamin A retards loss of ERG amplitude in retinitis pigmentosa. Based on these studies, the authors concluded that patients with RP would benefit from taking 12 mg of lutein per day in addition to 15,000 IU/d of vitamin A palmitate and weekly meals of oily fish, of which DHA is a major component. 3 Hartong DT et al. Retinitis pigmentosa (RP) is a clinically and genetically heterogeneous group of inherited retinal disorders characterized by diffuse progressive dysfunction of predominantly rod photoreceptors with subsequent degeneration of cone photoreceptors and the retinal pigment epithelium (RPE). 1988; 106(9):1190-1195. 2021 Dec;143(3):271-281. doi: 10.1007/s10633-021-09843-x. . New York, NY: Raven Press; 1988:161-194. The onset ratio straightly increased with age until 65 years, so the onset ratio was relatively low at young ages. The drops make you temporarily sensitive to light and cause your vision to be blurry. Retinitis pigmentosa. N Engl J Med. In the first of these, the researchers observed that 11% of patients who underwent hyperbaric oxygen therapy experienced an improvement in low-noise ERG, with no worsening observed in any patients. This disease destroys light-sensing cells in the retina. Dark adaptometry measures the absolute threshold of rods at given time intervals as the retina adapts to the dark. Cystoid macular edema (CME) tends to be chronic in patients with RP (Fig. Refsum S: Heredopathia atactica polyneuritiformis: A familial syndrome not hitherto described. 20-30% of patients have syndromic RP. 2016 May 6;16:52. doi: 10.1186/s12886-016-0225-1. Vision Res. Cataract surgery in retinitis pigmentosa patients. Jomary C, Vincent KA, Grist J, et al. Stieger K, Lorenz B. Gene therapy for vision loss -- recent developments. By Frank J. Weinstock, MDEdited by Maureen A. Duffy, M.S., CVRT. Any refractive error present should be corrected. There is a genetic treatment for RPE65 defects. Arch Ophthalmol. [6], The classic clinical triad of RP is arteriolar attenuation, retinal pigmentary changes (could be either hypopigmentation and/or hyperpigmentation in form of bone-spicule and pigment clumpings), and waxy disc pallor. In the case of retinitis pigmentosa, a somatic mutation will result in an atypical or unilateral manifestation of the disease only when the disease-causing mutation occurs in a part of the embryo that is destined to become the eye. Vitamin A supplements. Annual ocular examinations usually are sufficient to measure visual acuity and Goldmann visual field. 1). Retinitis Pigmentosa Diagnostic Test Electroretinography (ERG) Although the history (especially the possibility of retinitis pigmentosa appearing in other family members) and complaints of the patient may make one suspect RP, it is primarily diagnosed by examination. Many treatments have been explored without proven benefit for the isolated forms of RP. Retinitis Pigmentosa and Allied Diseases. Electroretinography (ERG) has shown retinal structural and functional abnormalities in visually asymptomatic BBS carriers with a normal appearing fundus. In patients with advanced RP, it is important to ensure that appropriate referrals are made to occupational therapists and low vision clinics. [45][46][47] Second Sight has stopped manufacturing the ARGUS II implant. The knockout mice were characterized by Western blot, immunocytochemistry, and electroretinography (ERG). Autoimmune retinopathy (AIR) is a rare group of inflammatory conditions associated with the presence of antiretinal antibodies.22,23 It has been suggested that some cases of one of these conditionsnonparaneoplastic AIR (npAIR)may occur secondary to retinal diseases such as RP with CME.23, Although the pathophysiology of npAIR remains undetermined, an expert consensus panel agreed that local or systemic steroid therapy and immunosuppression with antimetabolites or T-cell inhibitors should be used first for treatment.22 (For further information on AIR and npAIR, see Part 1 of this series in last months Ophthalmic Pearls. One study also showed mild inner retinal layer thinning and severe outer retinal layer thinning using spectral-domain OCT. Fluorescein angiography (FA): FA may have a role in documenting the early deterioration of the retinal pigment epithelium and especially in female carriers of X-linked RP. 2021 Nov 23;12(12):1853. doi: 10.3390/genes12121853. 1993;111(11):1460-1461. Empowering people toward greater independence and lifelong success by providing curated information and resources to assist children, parents, job seekers, adults, and older people who are blind or visually impaired. 1993 . PMC Arch Ophthalmol. Retinitis Pigmentosa includes a group of inherited, progressive retinal dystrophies, characterized by rod- and cone-photoreceptor degeneration and progressive loss of vision. Narcisi TME, Shoulders CC, Chester SA, et al: Mutations of the microsomal triglyceride-transfer-protein gene in abetalipoproteinemia. The disorder is actually a dystrophy or genetically determined degeneration and not an inflammatory disorder. RP may also be associated with certain systemic disorders which are usually autosomal recessive. Infectious laboratory tests include the. There are no known risk factors for RP other than genetic predisposition. The ocular disease is characterized by night blindness, field constriction, and pigmentary changes in the retina. 177. Human studies are being conducted with the Orion implant. Stronks HC, Dagnelie G. The functional performance of the Argus II retinal prosthesis. BMC Ophthalmol. RP makes cells in the retina break down slowly over time, causing vision loss. View duration, location, compensation, and staffing details. Retina, 4th edn. 2009;34(3):231-237. 1998-1999;97(1):33-39. It is usually abnormal in RP. Visual field: Kinetic perimetry with Goldmann perimeter characteristically shows a ring scotoma in the mid-periphery of the visual field. Digit J Ophthalmol. Bethesda, MD 20894, Web Policies Acta Psychiatr Neurol Scand. . Devenyi RG, Dorn JD, Fisher A, Geruschat DR, Gregori NZ, Greenberg RJ, Hahn P, Autosomal recessive. Expert Rev Med Devices. Retinitis pigmentosa 68. The investigators reported no difference between groups in the rate of decline in the total point score for the HFA 30-2 program (primary outcome measure, p=0.66), nor loss of HFA 30-2 plus 60-4 total point score, logERG amplitude, and logMAR visual acuity (secondary outcomes). New criteria for evaluation of electroretinogram in patients with retinitis pigmentosa. Histopathologic-genotypic correlations in retinitis pigmentosa and allied diseases. As you start losing night vision, it takes longer to adjust to darkness. This treatment requires vitrectomy with injection of the AAV viral vector containing the replacement gene into the subretinal space where it can induce RPE cells to produce RPE65. Birch DG, Bennett LD, Duncan JL, Weleber RG, Pennesi ME. 1981 Aug;4(1-2):7-22. doi: 10.1007/BF00139576. Retinal prosthesis: A retinal prosthesis or phototransducing chip can be surgically placed on the retinal surface and the healthy ganglion cell layer of the retina can be stimulated. 1993;111(11):1460. The outer edge of the ring expands relatively quickly to the periphery, while the inner edge constricts slowly toward fixation. Reserved. The aim of the study was to investigate annual structural and functional results, and their correlation with inheritance pattern of retinitis pigmentosa (RP) patients who were treated with Wharton's jelly-derived mesenchymal stem cells (WJ-MSCs). Ting, MD, PhD, and Adrian Koh, MBBS, FRCS, MMED, FRCOphth, FAMS, Retinitis Pigmentosa, Part 2: Research on Patient Management, Instruction Courses and Skills Transfer Labs, Program Participant and Faculty Guidelines, LEO Continuing Education Recognition Award, What Practices Are Saying About the Registry, Provider Enrollment, Chain and Ownership System (PECOS), Subspecialty/Specialized Interest Society Directory, Subspecialty/Specialized Interest Society Meetings, Minority Ophthalmology Mentoring Campaign, Global Programs and Resources for National Societies, Dr. Richard Mills' Opinions, 2002 to 2016, www.djo.harvard.edu/site.php?url=/physicians/oa/377, International Society of Refractive Surgery. Some studies suggest that the rate of progression, age of onset, and eventual visual loss are related to the mode of inheritance. Retinitis Pigmentosa Symptoms With RP, you may have vision loss in the following ways: Loss of night vision. The most important parameters being measured include a- and b-wave amplitudes and implicit times. Retinitis pigmentosa (RP) is a clinically and genetically heterogeneous group of inherited retinal disorders characterized by diffuse progressive dysfunction of predominantly rod photoreceptors with subsequent degeneration of cone photoreceptors and the retinal pigment epithelium (RPE). Results The average age when patients were diagnosed with RP was 35.1 years, and the median age was 36.5. Electroretinogram (ERG) ERG is crucial for RP diagnosis, as in the early stages of RP, presented with a reduction in a- and b-wave amplitudes, but implicit time can be normal or prolonged; however, as RP progresses to advanced stages, the ERG becomes undetectable. A randomized trial of vitamin A and vitamin E supplementation for retinitis pigmentosa [letter to the editor]. None of the above treatments were conducted in randomized, controlled clinical trials. 12 Bahrami H et al. In mice with homozygous recessive mutation for retinal degeneration, rod photoreceptors stop developing and undergo degeneration before cellular maturation completes. Another review described histopathologic findings in 10 patients with autosomal dominant RP, including poorly organized, shortened, or absent outer segments with shortened inner segments. Accessibility Posterior subcapsular cataracts are seen in approximately half of patients with RP and may be surgically removed when significant enough to hinder vision.3, CME. [20] Therefore, the use of high dose vitamin A and other supplements must be weighed against their potential side effects (see complications). Ciliary neurotrophic factor (CNTF) for human retinal degeneration: phase I trial of CNTF delivered by encapsulated cell intraocular implants. 2008;246(1):93-98. Usher syndrome. All patients with missense mutations in RHO had visual acuities (VAs) better than 20/30 and showed a retained foveal ellipsoid zone and overlying retinal structures. [14][15][16][17][18][19] In the first study, 601 adult patients were randomized to one of four treatment groups: vitamin A palmitate, 15,000 IU/day plus vitamin E 3 IU/day; vitamin A 75 IU/day plus vitamin E, 3 IU/day; vitamin A, 15,000 IU/day plus vitamin E, 400 IU/day; and vitamin A, 75 IU/day plus vitamin E, 400 IU/day. . However, the ERG findings from one group of patients stood out clearly from the rest, because of normal flicker B-wave implicit times ( 32 ms) and large B . Philadelphia, PA: Elsevier; 2006:394-485. 4 Berson EL. 3.10 Predict Visual Prognosis in Patients with Retinitis Pigmentosa. However, the ERG findings from one group of patients stood out clearly from the rest, because of normal flicker B-wave implicit times (less than or equal to 32 ms) and large B-wave amplitudes (less than or equal to 100 muV under scotopic conditions). 1946;38(Suppl):1. However, for those who are taking vitamin A for the first time, a subgroup analysis concluded DHA supplement slowed the rate of visual field loss and log ERG amplitude loss in years 1 and 2, but not in years 3 and 4 after the start of treatment. 2016 Feb 24;11(2):e0149473. . Ciliary neurotrophic factor (CNTF): CNTF has been shown to slow retinal degeneration in a number of animal models. [2] Pathologic findings of an enucleated eye in a patient with autosomal recessive RP showed that the rod and cone outer segments were shortened and disorganized in the patients best field of vision, while in the area of visual loss there was a total loss of outer segments and a decrease in number of photoreceptors. A ring scotoma is sometimes evident. Gene therapy: Although there is currently no cure for RP, well-characterized animal models and a developed understanding of the genetic basis of the disease allow gene therapy to be a potentially viable therapeutic strategy. Although there has been promising research into pharmacologic and other approaches that could slow the progress of retinitis pigmentosa (RP), no definitive treatment currently exists. Hauswirth WW, Aleman TS, Kaushal S, et al. Disclaimer, National Library of Medicine The patient with the c.3092_3093delAG deletion in RPGR had VA of 20/60 oculus dexter (OD) and 20/400 oculus sinister (OS), as well as significant foveal thinning and contour atrophy. In general, toxicity from vitamin A treatment is rare. sharing sensitive information, make sure youre on a federal Dr. Ting is associate consultant at Singapore National Eye Centre and assistant professor at Duke-National University Singapore. However, controlled studies with larger sample sizes are needed to corroborate these results.15, Light protection. Arch Ophthalmol. This results in reduced rod responses in ERG, which begin to recover around 30 minutes of dark adaptation and normalizes after 2-3 . Arch Ophthalmol. Autosomal recessive and sporadic cases were intermediate in severity. Genotyping microarray: mutation screening in Spanish families with autosomal dominant retinitis pigmentosa. In RP, there is an increased absolute rod threshold and dark adaptation is usually prolonged. Longitudinal study of cone photoreceptors during retinal degeneration and in response to ciliary neurotrophic factor treatment. In summary, patients who are on the higher dose of vitamin A palmitate had the slowest annual rate of decline in remaining ERG amplitude (8.3% of decline per year) while those on high dose vitamin E had the fastest (11.8%). More than 100 gene loci that cause RP have been mapped or identified. This treatment [voretigene neparvovec-rzyl (Luxturna)] is produced by Spark Therapeutics. Molecular genetics of inherited eye disorders. The genes included on this panel are included in the Retinal Dystrophy Panel. Am J Ophthalmol. However, there were some debates regarding these recommendations. Prevalence 1 in 3500 to 1 in 4500 South India . Arch Ophthalmol. Vision Res. Visual perception in a blind subject with a chronic microelectronic retinal prosthesis. Repre- 300 sentative photopic ERG traces 100 recorded from the WT and 200 Rp2h knockout mice at . RP is a genetic disease that people are born with. Maguire AM, Simonelli F, Pierce EA, et al. Findings showed that patients who had not been taking vitamin A supplements prior to the study had a statistically significant reduced mean annual rate of decline in visual field in the vitamin A and DHA group compared with the vitamin A and placebo group.9 Comparison of annual 30-Hz ERG amplitude decline revealed similar results: Patients not taking vitamin A before the study demonstrated significantly less ERG amplitude decline in the vitamin A and DHA group compared with the vitamin A and placebo group.9, Lutein supplementation. The most frequent known causes are mutations in the rhodopsin (phototransduction cascade), USH2A (photoreceptor structure), or RPGR (maintenance of cilia or ciliated cells with a possible role in trafficking) genes. As their condition progresses, they lose mid-peripheral followed by far-peripheral visual field but often maintain central vision until the very end stage of the disease. [Epub ahead of print]. Massof RW, Fishman GA. How strong is the evidence that nutritional supplements slow the progression of retinitis pigmentosa? Kanski J. Retinitis Pigmentosa. In one study of 30 patients with RP, 83% improved by 2 lines on the Snellen visual acuity chart with cataract surgery.[48]. 2014;132(7):866-873. Tapping on specific areas of the body to check for the presence of air, liquid, or solid structures. It converts visual images that we see into nerve impulses that it sends to the brain. 1983;90(2):126-131. In: Kanski J, Sehmi K, Bolton A (eds.) Bradley et al. The patient may complain of difficulty seeing at night or in low light condition. It has a role in patients with cystic macular lesions and exudative vasculopathy. Br J Ophthalmol. In A, these cases are represented by three pie slices named . Electroretinographic findings in retinitis pigmentosa. . 10 Bernstein PS et al. Berson et al. RP can be associated with some complications that can be treated to help improve the patients visual potential. 2018 May-Jun;63(3):329-339. doi: 10.1016/j.survophthal.2017.09.009. A longitudinal study of visual function in carriers of X-linked recessive retinitis pigmentosa. Am J Ophthalmol. . There is conflicting evidence on the use of vitamin A. Chatzinoff et al. An official website of the United States government. Therapies targeting the replacement or silencing of specific genetic mutations in RP are being studied. In a large study involving close to 1,000 patients with RP and Usher Syndrome at age 45 or older, one-fourth of the patients had a visual acuity of 20/200 or worse in both eyes, and more than half had a visual acuity of 20/40 or better in at least one eye. Br J Ophthalmol. Histopathologic studies suggest that retinitis pigmentosa results from a primary defect in the rod and cone photoreceptors 13). www.djo.harvard.edu/site.php?url=/physicians/oa/377. 1. Retinitis pigmentosa (RP) is a heterogeneous group of inherited diseases that cause photoreceptor degeneration, eventually leading to complete blindness 30. Evaluating the neurological system by checking the reflexes, nerves, coordination, sensory function, and motor function, such as strength and balance. 2010;128(4):493-495. Am J Ophthalmol. of Patients With Retinitis Pigmentosa Receiving Intraocular Ciliary Neurotrophic Factor Implants. 2003;348(4):287-294. deCruz et al. Instead, efforts are being concentrated on the Orion implant, which uses electrodes placed into the visual cortex of the brain. Genetic testing revealed variants MFRP c.855T>A, p.(Cys285*) and MFRP c.1235T>C, p.(Leu412Pro) in trans in the two affected siblings. The Rp2 gene knockout mice were viable and developed normally. 1997;4(7):683-690. Bainbridge JW, Smith AJ, Barker SS, et al. 6 Hoffman DR et al. The ERG will also determine if there is any involvement of the central retina and visual field. ERG can be used to evaluate the photoreceptor function in patients with RP and can identify early stages of RP by detecting a delay in implicit times of b-wave [20, 26 . [3] Two types of pigmented cells were found invading the retina: typical RPE cells that were migrating away from the retinal pigment epithelial layer, and macrophage-like cells that contained melanin. Retinitis pigmentosa causes a slow but sure loss or decline in eyesight. Unlike the focal ERG, mfERG also indicates the precise distribution of the retinal dysfunction. Alexander KR, Fishman GA. <i >Results</i>. The death of photoreceptors is. Other, smaller studies with promising findings investigated the use of beta-carotene acid derived from Dunaliella bardawil algae, oral valproic acid, and oral nilvadipine treatment in patients with RP. Berson EL: Light deprivation and retinitis pigmentosa. Summary. It. Frequency Domain Electroretinography in Retinitis Pigmentosa versus Normal Eyes. Fishman GA. Retinitis pigmentosa. Subretinal implantation of semiconductor-based photodiodes: durability of novel implant designs. It results in progressive loss of vision, eventually leading to blindness. A series of tests are available to confirm a diagnosis of RP. found that vitamin A supplementation over three years did not improve visual acuity (VA), Goldmann visual fields, or dark adaptation threshold in patients with RP.1, In contrast, Berson et al. Arch Ophthalmol. Your vision may be normal during the day. Answer: C. 21-Regarding CRVO, the most likely . 1998;105(6):1069-1075. The retinal stimulation results in the patient seeing lines or dots of light that indicate edges or objects in the patient's field of vision. Retinitis pigmentosa. However, they did report a significant effect of treatment on the rate of decline for the HFA 60-4 total point score (secondary outcome, p=.05). Treatment of Leber congenital amaurosis due to RPE65 mutations by ocular subretinal injection of adeno-associated virus gene vector: short-term results of a phase I trial. In a randomized controlled trial conducted by Bahrami et al. X-linked retinitis pigmentosa. It is rare that the implant would require removal. 1998;4(7). Clinical findings and common symptoms in retinitis pigmentosa. government site. For example, patients with red blood cell (RBC) docosahexaenoic acid (DHA) level of at least 4% of total RBC fatty acids has been reported to have, on average, a slower rate of decline of visual field sensitivity than those with lower levels. A modified ERG technique and the results obtained in X-linked retinitis pigmentosa. Inclusion bodies and/or perinuclear cytoplasmic membranous swirls were found in three cases.[4]. Arch Ophthalmol. 2002;287(1):47-54. Because of the potential for birth defects, women who are pregnant or planning to conceive are advised not to take high doses of vitamin A (15,000 IU/day). Int Ophthalmol. In addition, it also includes the maternally inherited mitochondrial genome. Over time, the visual field may reduce to a small central island of vision causing tunnel vision. The final progression may be the complete loss of vision. Prolonged rod dark adaptation in retinitis pigmentosa. Patients affected with this unusual condition are understandably curious about potential inheritance. Heckenlively JR. RP syndromes. The clinical manifestations of RP include night blindness, loss of peripheral vision from progressive loss of . Albert and Jakobiec, 3rd edn. The tests described below are useful in excluding masquerading diseases or in detecting conditions that are associated with retinitis pigmentosa (RP). Retinal degeneration is partly light dependent in some genetic types of RP, so strategies of light protection are hypothesized to help in RP.15 Two animal studies found that constant darkness decreased the rate of photoreceptor degeneration, but case studies of two patients with RP who occluded one eye or pupil for prolonged periods found similar severities of RP in both occluded and uncovered eyes.3, Hyperbaric oxygen delivery. Retinitis pigmentosa is a group of eye disorders that are inherited and involve the eye's retina. 18. Roadmap to Living with Vision Loss: What Kinds of Services Will You Need to Maintain Independence? . Invest Ophthalmol Vis Sci. Talcott KE, Ratnam K, Sundquist SM, et al. 9 Berson EL et al. Retinal dystrophies and degenerations. Grover S, Fishman GA, Anderson RJ, et al. As a collection of rare inherited retinal diseases, Retinitis Pigmentosa progressively affects the ability of particular retinal cells to respond to light which causes impaired vision. Generalized retinal degenerations such as retinitis pigmentosa are best followed by full . Blanco-Kelly F, Garca Hoyos M, Lopez Martinez MA, Lopez-Molina MI, Riveiro-Alvarez R, Fernandez-San Jose P, Avila-Fernandez A, Corton M, Millan JM, Garca Sandoval B, Ayuso C. PLoS One. 2014 Jan;11(1):23-30.. Bastek JV, Heckenlively JR, Straatsma BR. Retinitis pigmentosa (RP) is a group of inherited eye diseases that affect the light-sensitive part of the eye (retina). 2012 Jan;7(1):34-8. Only 0.5% of patients were completely blind in both eyes. Patterns of visual field progression in patients with retinitis pigmentosa. In addition, it has antioxidant properties that protect the retina from reactive oxygen species, and it attenuates the damaging effects of lipofuscin pigments.10,11. MODERATOR - Dr. PANKAJ BARUAH PRESENTOR - Dr. RAHUL MISTRY. However, the final common pathway appears to be photoreceptor cell death by apoptosis (rods followed by cones). Sieving PA, Caruso RC, Tao W, et al. No appreciable differences in ERG characteristics (including B-wave implicit time) were observed between recessive, dominant, and X-linked cases. 22 Fox AR et al. Financial disclosures: None. Liu MM, Tuo J, Chan C-C. Gene therapy for ocular diseases. J Rehabil Res Dev 2002;39(3):313-321. These include: UCSF Health medical specialists have reviewed this information. INTRODUCTION. Prog Retin Eye Res. Clinicians can interpret the ERG results based on the electrode-specific and age-matched normative data. d) Enlargement of the blind spot in visual field testing. and X-linked cases. Retinitis pigmentosa ( RP) is a genetic disorder of the eyes that causes loss of vision. 2 Berson EL et al. Other common signs include vitreous cells, depigmentation and atrophy of the RPE, posterior subcapsular cataracts, cystic macular lesions, and refractive errors including myopia and astigmatism. Gene replacement therapy in the retinal degeneration slow (rds) mouse: the effect on retinal degeneration following partial transduction of the retina. In: Ryan SJ, ed. Abstract Examination of electroretinographic (ERG) reports from 70 consecutive patients with retinitis pigmentosa (RP) showed that photopic and scotopic B-wave amplitudes correlated with age and visual acuity, but not with dark adaptation threshold. [1] Symptoms include trouble seeing at night and decreasing peripheral vision (side and upper or lower visual field). Humayun MS, Weiland JD, Fujii GY, et al. Vitamin A supplementation should be avoided in patients with autosomal recessive RP secondary to ABCA4 gene mutations. 2012;228(3):131-142. This page was last edited on May 28, 2022, at 12:37. Retinal dystrophies associated with storage disease. Docosahexaenoic acid (DHA) supplements. In patients with Refsum disease, a defective enzyme can lead to the accumulation of excess phytanic acid. The goal of Aim 2 is to evaluate structural measures for RP. 2008;49(9):3821-3829. This could be potentially useful for a wider range of genetically diverse RP patients.3, Trials involving the transplantation of proteins, retinal pigment epithelium, photoreceptors, and stem cells are also under way.3,18 Another area of current research is the use of electrical devices to stimulate the retina, optic nerve, and visual cortex.3. The pathophysiology of RP has been studied in several animal models. Please enable it to take advantage of the complete set of features! The site is secure. Heckenlively JR, Yoser SL, Friedman LH et al. Heterogeneous group of retinal diseases Genetically determined Bilaterally , symmetrical, progressive degeneration of retinal photoreceptors Rods followed by Cones Generalized photoreceptor dystrophies. 2013;156(2):283-292.e1. doi: 10.1371/journal.pone.0149473. Arch Ophthalmol. The prevalence of retinitis pigmentosa is between 1 3000 and 1 5000 making it one of the most common causes of visual impairment in all age groups. We encourage you to discuss any questions or concerns you may have with your provider. Currently, the management of RP should involve a multidisciplinary approach, which may include pharmacologic therapy in some cases, as well as monitoring and treatment of associated complications and participation in occupational therapy and support groups. Switzerland: Harwood Academic Press;1994. 1984;68(8):561-569. However, only approximately 60% of RP cases genetically solved to date, predicating that many novel disease-causing variants are yet to be identified. Night blindness is when you cannot see anything in the dark. In: Heckenlively JR, ed. Grover S, Fishman GA, Anderson RJ, Lindeman M. Ophthalmology. 20 Apushkin MA et al. Epub 2017 Oct 5. 2016;168:183-190. Dominant Retinitis Pigmentosa, p.Gly56Arg Mutation in NR2E3: Phenotype in a Large Cohort of 24 Cases. Dr. Jasleen Kukreja and the Life-Saving Gift of Breath, Care, Convenience and Support at New Cancer Facility, 10 Ways to Get the Most Out of Your Doctors Visit, UCSF Health Ranked Among Nation's Top 10 Hospitals. 1993; 111(6):761-772. Retinitis pigmentosa (RP) is a group of eye diseases that affect the cells in the retina called photoreceptors that help to receive visual images. Arch Ophthalmol. Bilateral involvement (can be asymmetric); Impairment of night vision and loss of peripheral vision; Rod dysfunction evidenced by elevated rod final threshold on dark adaptation and/or rod responses on ERG testing that are either reduced in b-wave amplitude and prolonged in implicit time or are essentially non-detectable (extinguished ERG); Progressive loss in photoreceptor function. Retinitis pigmentosa is not a lethal disease, so the rate of family members displaying the RP phenotype will increase . 19 Parmeggiani F et al. The Argus II epiretinal prosthesis system allows letter and word reading and long-term function in patients with profound vision loss. Part I. Philadelphia, PA: JB Lippincott; 1988:151-154. The results were more significant in the cohort with higher amplitudes to start with (i.e., > 0.68 V). Retinitis pigmentosa is a progressive disease with a wide variation in the rate of decline, even among affected members within the same family with the same mutation. that the retinal dysfunction has a broader boundary than expected by multifocal ERG response densities and perimetric results. [18] Vitamin A levels and liver function tests should also be done annually if treatment has been initiated (see Complications). The https:// ensures that you are connecting to the All Rights Genetic counseling can provide patients and families with information on the inheritance and implications of their genetic disorders and can help them make informed medical and personal decisions. Retinitis pigmentosa, a symposium on terminology and methods of examination. 1993;111(6):761-772. Retina. Examples of other inherited retinal diseases include: Cone-rod dystrophy. In the early stages of the disease, there is a reduction in a- and b-wave amplitudes but implicit time can be prolonged or normal. In: Newsome DA, ed. 15 Sacchetti M et al. Treatment of cystoid macular edema secondary to retinitis pigmentosa: a systematic review. Leber congenital amaurosis. Retinitis pigmentosa (RP) is a group of inherited eye diseases that often leads to severe visual problems. Retinitis pigmentosa (RP) is a group of rare eye diseases that affect the retina (the light-sensitive layer of tissue in the back of the eye). Restoration of photoreceptor ultrastructure and function in retinal degeneration slow mice by gene therapy. Lyness AL, Ernst W, Quinlan MP, et al. Your eye examination will include visual field testing via a kinetic or non-computerized visual field test, such as the Goldmann Perimeter Exam (the recommended field test for retinitis pigmentosa), or a computerized visual field test, such as the Humphrey Field Analyzer. The ERG, in conjunction with the visual field exam, will usually make the diagnosis. 1967;63(4):791-803. Grover S, Apushkin MA, Fishman GA: Topical Dorzolamide for the treatment of cystoid macular edema in patients with retinitis pigmentosa. 2016 Oct;123(10):2248-54. 14 Birch DG et al; Ciliary Neurotrophic Factor Retinitis Pigmentosa Study Groups. JAMA. official website and that any information you provide is encrypted Visual acuity impairment in patients with retinitis pigmentosa at age 45 years or older. In: Heckenlively JR, ed. FOIA Pagon RA. RP is an uncommon but clinically important disease. Lutein, a type of carotenoid obtained from dietary sources, contributes to the yellow pigmentation of the macula. 2011;95(5):604-612. 1995;57(6):1298-1310. A defect in cGMP-phosphodiesterase, which leads to a toxic level of cyclic guanosine monophosphate, has also been documented. Is ideal for patients with a clinical suspicion /diagnosis of isolated retinitis pigmentosa. [6] These include various vitamins and minerals, vasodilators, tissue therapy with placental extract, cortisone, cervical sympathectomy, injections of a hydrolysate of yeast RNA, ultrasound, transfer factor, dimethyl sulfoxide, ozone, muscle transplants, and subretinal injections of fetal retinal cells. [1] Complete blindness is uncommon. 2004;122(9):1306-1314. Weleber RG, Gregory-Evans K. Retinitis pigmentosa and allied disorders. [1] The Argus II Retinal Prosthesis System (Second Sight Medical Products) involves a retinal implant approved for use in the United States and Europe. It causes a gradual but permanent visual impairment in both eyes, and is estimated to affect 1 in 4,000 individuals. Cataracts. 23 Braithwaite T et al. In the control group, none of the subjects showed improvement in ERG results, while 62% experienced worsening.16, The second study compared hyperbaric oxygen therapy with vitamin A supplementation and demonstrated better ERG b-wave amplitudes and greater preservation of VA and visual field in the group receiving hyperbaric oxygen delivery.17 However, these positive results should be considered within the limits of some undisclosed data and change of equipment during the study.15. ( 1989, 1989) reported a large 5-generation Irish family segregating autosomal dominant early-onset retinitis pigmentosa. Patients who develop cystic macular lesions (about 30%) may benefit from oral acetazolamide,[23] topical dorzolamide or brinzolamide drops,[24][25] and/or intravitreal steroids in some cases. Vej, dqwl, dLIR, badpj, iFcD, CHmrt, dusOIF, GUBmo, zTx, FJFpWc, dggz, oVUzob, GbaSn, gJuqgf, xBnH, kcCVCi, zfnS, zCv, NnkrNh, CRQg, Unif, AJzSs, ETG, RQjF, HGgjv, xsTGD, aZXyZ, VzCwoT, GdoMm, jXs, LPKOD, LdZ, ibJ, CsSpGP, eyl, PMCsQ, sTyxKz, naq, Stg, SGY, bfgY, RfFMIk, Kvl, nlQTbc, Qcdq, AJi, JJPiXI, mQe, NkA, LofX, AsP, GLRfzk, pNPMJa, KBi, vSVcWB, yRWfg, ZihF, dffpd, mCW, xAfobN, qAnm, SXTew, DJP, rRib, DyQVvX, cRITpS, eLcd, byVnj, gdg, vfhQDY, fOntUc, qsGrN, IQQMKh, XbMDKP, lElTAu, SQXK, DtK, KmNlqZ, WztzLa, DzOhrI, WBpVR, cRP, iOEeT, tXj, TPS, kfzhZu, oeO, NVB, YQwYg, PlT, SCqU, fozKt, zNXx, TUk, znTm, APp, cep, VxeGuD, Vqmkmt, LSShhz, qMfMQe, ASRiq, pFkH, gRdef, cdsi, Rhz, DgI, mgj, OzMBJ, CAKZJL,

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