Der Ophthalmologe

, Volume 109, Issue 2, pp 112–120 | Cite as

Pharmakologische Ansätze in der Therapie erblicher Netzhautdegenerationen

Leitthema
First Online:

Zusammenfassung

In dieser Übersichtsarbeit wird der aktuelle Stand der medikamentösen Therapie erblicher Netzhautdegenerationen beschrieben. Während eine kausale Therapie trotz zunehmendem Verständnis der Pathomechanismen der Erkrankungen bislang nicht existiert, können Komplikationen wie zystische makuläre Läsionen oder ein zystoides Makulaödem mit lokalen und systemischen Karboanhydrasehemmern, ggf. ergänzt durch Steroide, günstig beeinflusst werden. Der überwiegende Effekt ist eine Netzhautdickenreduktion, die aber nicht immer mit einem Visusanstieg einhergeht. Eine engmaschige Kontrolle mittels optischer Kohärenztomographie ist notwendig. Mögliche neuroprotektiv wirkende Substanzen (Valproinsäure, „ciliary neurotrophic factor“ und Ca2 + -Kanal-Antagonisten) oder Nahrungsmittelergänzungen (Vitamin A, Lutein, synthetische Retinoide und Docosahexaensäure) können im Einzelfall einen günstigen Effekt auf den Krankheitsverlauf (z. B. Reduktion der Progression des Gesichtsfeldausfalls oder einzelner elektrophysiologischer Parameter) haben. Die Wirkungen und mögliche Nebenwirkungen hängen z. B. bei der Vitamin-A-Substitution nicht nur vom Phänotyp (z. B. Retinitis pigmentosa), sondern auch von der genetischen Ursache der Erkrankung ab. Studien zur Anwendung im Kindesalter fehlen vollständig.

Schlüsselwörter

Retinitis pigmentosa Zystoides Makulaödem Netzhautdystrophie „Ciliary neurotrophic factor“ Docosahexaensäure 

Pharmacological concepts to treat hereditary retinal degenerations

Abstract

This article reviews the current pharmacological strategies to treat inherited retinal degeneration. To date there is no causal therapy despite growing knowledge of the particular pathomechanisms. However, treatment is available for complications, such as cystic macular changes and cystoid macular edema. To reduce retinal thickness systemic or topical carboanhydrase inhibitors can be applied and in rare cases combined with steroids when indicated, however reduction of retinal thickness is not always accompanied by improvement of visual acuity. Regular follow-up with optical coherence tomography is required. In some cases, potentially neuroprotective agents (valproic acid, ciliary neurotrophic factor and Ca2+  channel inhibitors) or food supplementation (vitamin A, lutein, synthetic retinoids and decosahexaenoic acid) may have a positive impact on disease progression (e.g. reduction in progression of visual field loss or individual electrophysiological parameters). However, beneficial effects and side effects, e.g. of vitamin A substitution, depend not only on the disease phenotype (such as retinitis pigmentosa) but also on the actual genotype. Furthermore, no data are available regarding the application of pharmaceuticals in the pediatric population.

Keywords

Retinitis pigmentosa Cystoid macular edema Retinal dystrophies Ciliary neurotrophic factor Docosahexaenoic acid 
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Notes

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  1. 1.Universitäts-Augenklinik FreiburgFreiburgDeutschland
  2. 2.Universitäts-Augenklinik RegensburgRegensburgDeutschland

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