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      Características clínicas y evolución de la retinosis pigmentaria en adolescentes Translated title: Clinical characteristics and evolution of retinitis pigmentosa in adolescents

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          Abstract

          Objetivo: Identificar las características clínicas y la evolución de la retinosis pigmentaria en la adolescencia. Método: Estudio descriptivo y prospectivo en adolescentes entre 10 y 14 años de edad (grupo I) y entre 15 y 19 años, (grupo II), del sexo masculino y del femenino, atendidos en Centro de Referencia Nacional de Retinosis Pigmentaria- Pediátrico entre 2002 y 2006. Se observó herencia, síntoma inicial y edad, tiempo transcurrido y enfermedades generales concomitantes y los signos del FO por biomicroscopia y oftalmoscopia, perimetría y ERG al inicio, anual y al final. Se clasificó el estadio en 4 etapas atendiendo a parámetros como agudeza visual con corrección. y CV. Resultados: Entre los 42 adolescentes predominaron los varones y el color de la piel blanca. El DVP estuvo presente en 12 ojos (14,2 %), las opacidades del cristalino en 61 ojos (71,4 %), principalmente en el grupo 2. Al final del estudio, según la agudeza visual y CV, 32 adolescentes (76,2 %) tuvieron con evolución no progresiva, en 5 (11,9 %) fue medianamente progresiva y en 5 (11,9 %) fue muy progresiva pues hubo marcado empeoramiento de la función visual. Conclusiones: Las características de la retinosis pigmentaria que se identificaron en los adolescentes se incluyen entre los 3 primeros estadios. La evolución durante 5 años no fue progresiva en la mayoría de los adolescentes de ambos grupos, lo cual induce a considerar que los cambios biológicos de esta etapa de la vida aparentemente no influyen en el curso de la retinosis pigmentaria.

          Translated abstract

          Objectives: Identify the clinical characteristics and evolution of retinitis pigmentosa in adolescents. Method: A prospective descriptive study of adolescents aged 10 to 14 years (group I) and 15-19 years (group II) of both sexes and treated at the National Reference Center of Pediatric Retinitis Pigmentosa from 2002 to 2006. Inheritance, initial symptoms, age, time elapsed and general concomitant diseases, FO signs by biomicroscopy and ophthalmoscopy, perimetry and ERG at the beginning, annually and at the end were all examined. Four stagings were set up according to parameters such as visual acuity with correction and CV Results: Males and Caucasians predominated in the 42 studied teenagers. DVP was present in 12 eyes (14.2), crystalline opacities were found in 61 eyes (71.4%) mainly in the group II. At the end of the study, according to the visual acuity and CV, 32 adolescents (76,2%) did not have progressive evolution of the disease, 5 (11,9%) had moderately progressive and 5 (11,9%) showed very progressive evolution since there was marked worsening of the visual function. Conclusions: The identified characteristics of retinitis pigmentosa in adolescents were within the three first stagings. Evolution was not progressive for 5 years in most of adolescents from both groups, which led us to consider that the biological changes in this period of life seem to have no influence in the course of retinitis pigmentosa.

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          Most cited references28

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          Problemas médicos de los adolescentes

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            Oftalmología Clínica

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              Epidemiology of retinitis pigmentosa in the Valencian community (Spain).

              The purposes of this study are to determine the frequencies of the different genetic forms of retinitis pigmentosa and to perform segregation analysis in the different genetic subtypes. Retinitis pigmentosa was diagnosed in 263 persons from 132 families. The frequency of the autosomal recessive type was the highest (31.8%) while the X-linked type was very rare (1.5%). The frequency of autosomal dominant type was 14.4% and the simplex cases constituted half of the total cases of RP registered in our community. In conclusion, in our population the high proportion of simplex cases and the low number of X-linked families are noticeable. The result of segregation analysis showed good agreement with expectation in autosomal dominant and autosomal recessive families but no more than 60% of all simplex cases were autosomal recessive. The proportion of sporadic cases was estimated statistically to be 39.9% of the total simplex cases.
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                Author and article information

                Journal
                oft
                Revista Cubana de Oftalmología
                Rev Cubana Oftalmol
                Editorial Ciencias Médicas (Ciudad de la Habana, , Cuba )
                0864-2176
                1561-3070
                December 2007
                : 20
                : 2
                Affiliations
                [01] Ciudad de La Habana orgnameHospital Docente Dr. Salvador Allende orgdiv1Centro de Referencia Nacional de Retinosis Pigmentaria Cuba
                Article
                S0864-21762007000200007 S0864-2176(07)02000207
                e6f6fd01-3cf2-420b-93dc-97198eeab995

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 20 July 2007
                : 21 April 2007
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 22, Pages: 0
                Product

                SciELO Cuba

                Categories
                TRABAJOS ORIGINALES

                Retinosis pigmentaria,adolescencia,ceguera nocturna,adolescence,Retinosis pigmentosa,night blindness

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