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Minerva Oftalmologica 2010 March;52(1):1-15


language: Italian

Clinical management of retinitis pigmentosa: un update

Parmeggiani F. 1, Chizzolini M. 2, De Nadai K. 2, Costagliola C. 3, Semeraro F. 4, Sebastiani A. 1

1 Clinica Oculistica, Università degli Studi di Ferrara, Ferrara, Italia 2 Centro di Riferimento per la Retinite Pigmentosa della Regione Veneto. Unità Operativa Autonoma di Oculistica, Ospedale di Camposampiero, Azienda ULSS 15 Alta Padovana, Padova, Italia 3 Dipartimento di Scienze della Salute, Università degli Studi del Molise Campobasso, Italia 4 Clinica Oculistica, Università degli Studi di Brescia, Brescia, Italia


The term retinitis pigmentosa (RP) indicates a heterogeneous group of genetic eye diseases in which the rods or the cones are prevalently damaged. In Caucasian populations, RP prevalence is variable reported in 1 case for each 3000-5000 individuals. RPs represent progressive tapeto-retinal dystrophies that are often related to severely limit visual performances. In the course of typical RP (rod-cone dystrophy), the affected individuals first experience defective dark adaptation or nyctalopia (night blindness), followed by reduction of the peripheral visual field and, sometimes, loss of central vision late in the course of the disease. In case of atypical RP (cone-rod dystrophy), the cone’s functionalities are more rapidly disrupted in comparison with the rod’s ones. Sometimes, people with RP do not become legally blind until their 40s or 50s and/or retain a certain level of sight for all their life. Others go completely blind from RP, in some cases as early as school childhood. The RP diagnosis relies upon documentation of progressive loss in photoreceptor function by electroretinography and/or visual field testing. There is currently no treatment that can effectively cure RP. However, in some case-series, the progression of the disease has been reduced by the intake of vitamin A palmitate or by the hyperbaric oxygen therapy. Moreover, in the most part of the patients affected by RP, the quality of vision can be significantly increased by the use of nanometer-controlled filters. Future clinical applications of the current experimental treatments will reliably involve encapsulated cell technology for the release of neurotrophic factors, gene therapy, artificial retinal implants and stem cells.

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