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      Retinitis Pigmentosa in Onitsha, Nigeria

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          Abstract

          Background:

          Retinitis pigmentosa describes a heterogenous group of progressive hereditary degeneration of the photoreceptor-retinal pigment epithelium complex with varying clinical manifestations. Although studies on the epidemiology of blindness and visual impairment in Nigeria have documented the contribution of retinitis pigmentosa, detailed study of the prevalence, incidence, and pattern of retinitis pigmentosa in Nigeria are few.

          Objectives:

          The aim of this study was to describe the relative frequency rate and pattern of retinitis pigmentosa among new patients at the Guinness Eye Center Onitsha Nigeria.

          Materials and Methods:

          Case files of new patients with clinical features of retinitis pigmentosa seen over a 6-year period were reviewed. Information on age, sex, and associated clinical features were extracted into a standard proforma and analyzed.

          Results:

          Thirty-seven (0.6%) of 5876 new patients had retinitis pigmentosa. There were 25 (67.6%) males and 12 (32.4%) females with an age range of 4–76 years; the median age was 33 years. Family history suggested autosomal dominant inheritance in 15 (40.6%) patients, autosomal recessive inheritance 4 (10.8%), X-linked inheritance 9 (24.3%), and 9 (24.3%) sporadic. Six (16.2%) patients were blind (acuity <3/60) and 22 (59.5%) patients had visual impairment (acuity <6/18). Eight (21.6%) patients had coexistent deafness (syndromic retinitis pigmentosa) including two (5.4%) cases of Usher syndrome.

          Conclusions:

          The relative frequency rate of retinitis pigmentosa among new patients in our hospital is low but the associated visual and otologic incapacitation are enormous. A prospective study incorporating molecular genetics would more exactly determine the inheritance pattern and the common genes of retinitis pigmentosa in Nigerians.

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          Most cited references28

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          Prevalence and causes of visual impairment and blindness among 9980 Scandinavian adults: the Copenhagen City Eye Study.

          To investigate the age-specific prevalence and causes of visual impairment and blindness in an epidemiologic study of an adult Scandinavian population. Population-based, cross-sectional study. The study population was composed of 9980 persons, ages 20 to 84, from the general population of Copenhagen, Denmark. This study is based on the third Copenhagen City Heart Study (CCHS III). Participants who reported visual impairment or blindness or had difficulty reading newspaper type and used prescribed eye medications were contacted from 1999 through 2000 and asked to complete a standardized interview concerning their ophthalmologic history. Verification of objective ophthalmologic data was done with a validated questionnaire response method. Best-corrected visual acuity in the better eye and primary causes of visual impairment and blindness. Visual impairment was defined as visual acuity worse than 20/40 but better than 20/200, and blindness was defined as visual acuity of 20/200 or worse. The age-standardized prevalence rates of visual impairment and blindness were 0.66% and 0.20%, respectively, and rose significantly with age (P<0.001). For persons 20 to 64 years, myopia-related retinal disorders, diabetic retinopathy, optic neuropathy, and retinitis pigmentosa were the most common causes of impaired vision. For persons 65 to 84 years, cataract was the most common cause of visual impairment, whereas age-related macular degeneration was the major cause of blindness. Visual impairment and blindness are strongly associated with increasing age, and the causes are determined by age. Among persons aged 20 to 64 years, an intervention for the predominating eye diseases might have some effect. Among those aged 65 to 84 years, cataract surgery could reduce visual impairment by one third.
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            Causes of blindness and visual impairment in Nigeria: the Nigeria national blindness and visual impairment survey.

            Determine causes of blindness and visual impairment among adults aged >or=40 years. Multistage, stratified, cluster random sampling with probability proportional to size procedures were used to identify a nationally representative sample of 15,027 persons >or=40 years of age. Distance vision was measured with a reduced logMAR tumbling E-chart. Clinical examination included a basic eye examination of all subjects and a more detailed examination of those who had presenting vision or=40 years who were enumerated, 13,599 (89.9%) were examined. In 84%, blindness was avoidable. Uncorrected refractive errors were responsible for 57.1% of moderate ( 40% over the next decade.
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              Age at onset curves of retinitis pigmentosa.

              To calculate age at onset curves of retinitis pigmentosa (RP) to resolve the difficulty in calculating the recurrence risk in a family. Retinitis pigmentosa is a common hereditary retinal disease that leads to blindness. It is a slow-onset disease, and family members of patients sometimes develop RP later. We studied 370 patients with typical RP. The age at onset was defined as when the patient's RP was diagnosed by an ophthalmologist. After confirmation that the age at onset came from normal distribution, we drew the age at onset curves. The average age when patients were diagnosed with RP was 35.1 years, and the median age was 36.5. The onset ratio straightly increased with age until 65 years, so the onset ratio was relatively low at young ages. The age at onset curves are quite simple and useful tools that facilitate counseling at an RP clinic. Without them, the recurrence risk would be misleading.
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                Author and article information

                Journal
                J West Afr Coll Surg
                J West Afr Coll Surg
                JWACS
                Journal of the West African College of Surgeons
                Wolters Kluwer - Medknow (India )
                2276-6944
                Apr-Jun 2020
                26 March 2022
                : 10
                : 2
                : 30-35
                Affiliations
                [1 ] Department of Ophthalmology, Nnamdi Azikiwe University, Awka, Nigeria
                [2 ] Centre for Eye Health Research and Training, Nnamdi Azikiwe University, Awka, Nigeria
                [3 ] Guinness Eye Center, Onitsha, Nigeria
                [4 ] Ebony Eye Specialist Clinic, Onitsha, Nigeria
                [5 ] Department of Otorhinolaryngology, University of Benin Teaching Hospital, Benin City, Nigeria
                Author notes
                Address for correspondence: Prof. Sebastian N. N. Nwosu, Guinness Eye Center, PMB 1534, Onitsha, Nigeria. E-mail: sabenwosu@ 123456gmail.com
                Article
                JWACS-10-30
                10.4103/jwas.jwas_65_21
                9089809
                35558569
                ff5fc861-66d6-45bf-804f-354b4f9dc7af
                Copyright: © 2022 Journal of West African College of Surgeons

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 28 December 2021
                : 30 January 2022
                Categories
                Original Article

                blindness,deafness,pattern,retinitis pigmentosa
                blindness, deafness, pattern, retinitis pigmentosa

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