Treatment of a retinal dystrophy, fundus albipunctatus, with oral 9-cis-{beta}-carotene.
Br J Ophthalmol
; 94(5): 616-21, 2010 May.
Article
em En
| MEDLINE
| ID: mdl-19955196
ABSTRACT
BACKGROUND Fundus albipunctatus is a retinal dystrophy caused by a mutation in the gene encoding 11-cis-retinol dehydrogenase which delays the recovery of rod photoreceptor cells from light stimulation leading to night blindness. A recent study of a mouse model of fundus albipunctatus treated with 9-cis-retinal showed an improvement in visual function and structure. METHODS Seven patients with fundus albipunctatus were given a daily food supplement of four capsules containing high-dose 9-cis-beta-carotene for 90 days. The subjects were tested before and after treatment by visual field and electroretinogram in both eyes. This non-randomised prospective phase I study was registered at http//www.clinicaltrials.gov (NCT00478530). RESULTS All patients showed significant improvements in peripheral visual field (mean deviation improved from -4.77+/-2.0 to -3.28+/-2.28, p=0.009, t test) and a highly significant improvement in rod recovery rates measured electroretinographically (maximal scotopic b-wave amplitude responses, improved from 197+/-49 muV to 292+/-48 muV, p<0.001, t test). No complications or side effects were observed. CONCLUSION Oral treatment with 9-cis-beta-carotene led to reversal of a human retinal dystrophy. This potential therapy is readily available and should be evaluated in retinal dystrophies of similar mechanisms such as various types of retinitis pigmentosa.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Degeneração Retiniana
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Vitaminas
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Oftalmopatias Hereditárias
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Beta Caroteno
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Suplementos Nutricionais
Tipo de estudo:
Clinical_trials
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Observational_studies
Idioma:
En
Revista:
Br J Ophthalmol
Ano de publicação:
2010
Tipo de documento:
Article
País de afiliação:
Israel