What is reticulitis in humans

Retinitis pigmentosa (RP)

Clinical picture

From the brochure "Retinitis Pigmentosa (RP) - What is it?", 6th edition 03/2016

Contents overview

introduction

  1. Features and course of retinitis pigmentosa
  2. Syndromes, special forms of retinitis pigmentosa and other retinal degenerations
  3. Cataracts and retinitis pigmentosa
  4. Inheritance
  5. Research and therapy

introduction

Retinitis pigmentosa (RP) is the name for a group of hereditary eye diseases that result in the destruction of the retina, the visual tissue at the back of the eye. The misnomer retinitis (it is not an inflammation of the retina) has nevertheless prevailed over the term retinopathia in medical parlance; the adjective pigmentosa describes the typical pigment deposits in the retina that are visible when examining the fundus.

Around three million people worldwide - around 30,000 to 40,000 in the Federal Republic of Germany - suffer from one of the various forms of RP. This currently incurable disease is one of the most common causes of vision loss in middle adulthood. RP is a hereditary disease that can be passed on to offspring. It is estimated that every 80th person carries an "unfavorably" modified RP gene, i.e. genetic information that can trigger the development of this retinal disease in gene carriers or their descendants.

The diseases of this group are characterized by the fact that night blindness usually occurs in adolescence or middle age, the field of vision is then narrowed, contrast and color vision, and later also the visual acuity deteriorate, so that the eyesight gradually deteriorates, not infrequently up to Blindness.

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The entire process is gradual or in batches and usually extends over decades. This development is associated with professional and private disadvantages and thus with a strong psychological burden.

The cause of these symptoms is a gradual decline of the light sensory cells (photoreceptors) of the retina of the eye, mostly initially the rods responsible for night and twilight vision, later also the cones located in the center of the retina, which are important for reading and color vision. Although the disease was already described in the middle of the 19th century, there is still - with a few exceptions - no possibility of surgically, medicinally or dieting to slow down or stop the process of death of the visual cells. Since knowledge of the disease, a large number of therapeutic attempts have been carried out, ranging from serious scientific approaches to quackery and largely unsuccessful.

The history of modern medicine shows that new and effective therapies can only be developed on the basis of scientific research and thus rational therapeutic approaches can be found.

Only in the case of a few rare special forms of RP has it been possible to find treatment options that can bring the process to a standstill with targeted measures.

Basic research has made considerable progress in recent years. Molecular genetics, in particular, is required to use its modern methods in the case of hereditary diseases - including RP - to track down the disease-causing changes in the genetic material and thus contribute to clarification and, where possible, to a therapeutic approach. In other areas of research, too, approaches can be identified that could lead to treatment in the medium term.

The PRO RETINA Germany e. V. and the other national associations working in the international RP movement have made research into RP - in addition to social care for those affected - a priority and hope to come considerably closer to this goal in the next ten years.

With this information leaflet, PRO RETINA Deutschland e. V. inform those affected and their relatives as well as the public about the manifestations and symptoms of this still incurable disease, the possibilities of personal coping and hopeful research approaches aimed at therapy.

We would like to take this opportunity to thank Dr. D. Besch, University Eye Clinic Tübingen, Professor Dr. K. Rüther, Charité Humboldt University Berlin, Dr. E. Apfelstedt-Sylla, Stuttgart, and Professor Dr. A. Gal, Institute for Human Genetics, Hamburg, for their helpful suggestions for corrections and detailed advice.

The board of directors

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Last changed on 11/12/2018 11:25 AM