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Retinitis Pigmentosa

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Hereditary Chorioretinal Disorders

Part of the book series: Retina Atlas ((RA))

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Abstract

Retinitis pigmentosa (RP) is a heterogeneous group of disorders characterized by the degeneration of photoreceptor cells and the retinal pigment epithelium, leading to profound vision loss or blindness. The prevalence of RP is approximately one in every 4000 individuals worldwide (Hartong et al. 2006). In 1836, Bernhard von Langenbeck used the term melanosis retinae to describe the pigmented condition of the retina during a postmortem examination (Langenbeck 1836). Later, in 1838, Friedrich von Ammon published drawings of widespread pigmentation based on pathological studies of the eye but did not correlate the condition to night blindness (Ammon 1838). After Helmholtz invented the ophthalmoscope in 1851, van Trigt in 1853 and Ruete in 1854 identified this disease in living subjects and linked it to visual symptoms (van Trigt 1853; Ruete 1855), which was ultimately named retinitis pigmentosa in 1857 by Franciscus Donders (Donders 1857). Even though there are no inflammatory processes in RP, the same name is still used today. To date, over a hundred years later, several treatment options have been proposed for patients with RP such as gene therapy, stem cells, and retinal prosthesis. However, long-term outcomes still need further investigation.

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Chou, HD., Wu, AL., Cheng, YC., Wang, NK. (2020). Retinitis Pigmentosa. In: Cheung, G. (eds) Hereditary Chorioretinal Disorders. Retina Atlas. Springer, Singapore. https://doi.org/10.1007/978-981-15-0414-3_1

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