Abstract

BackgroundStargardt’s disease (STGD) and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite way, by diet.MethodsDietary profile was assessed in 24 patients with STGD and in 56 patients with RP. We documented in only 6 out of 24 (25 %) STGD patients a daily intake of vitamin A within the recommended range while 14/24 (58.3 %) reported a high daily intake and 4/24 (16.7 %) showed a low daily intake. With regard to RP, 4/56 (7.1 %) reported to be within the recommended range, 37/56 (66.1 %) reported high daily intake and 15/56 (26.8 %) showed low daily intake of vitamin A.ResultsInterestingly, STGD patients with low vitamin A intake (<600 µg RAE/day) showed significantly better visual acuity with respect to those introducing higher intake of vitamin A.ConclusionThe present study suggests insuitable nutrient intakes among patients with STGD and RP, especially for daily intake of vitamin A. The results may be used to provide tailored nutritional interventions in these patients.

Highlights

  • Stargardt’s disease (STGD) and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite way, by diet

  • Dietary profile Dietary pattern of the study population is described in Tables 1 and 2, for STGD and RP respectively

  • By comparing the dietary patterns with the dietary reference intakes reported by the Italian Society of Human Nutrition (Italian Society of Human Nutrition, [15]) in order to prevent the major chronic diseases we can observe some divergences from the recommendations

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Summary

Introduction

Stargardt’s disease (STGD) and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite way, by diet. Stargardt’s Disease (STGD) and Retinitis Pigmentosa (RP) are genetic-based relevant ocular diseases that may be affected, in opposite way, by vitamin A intake and other nutrients [3,4,5]. In an animal model of STGD vitamin A supplementation has been shown to accelerate the accumulation of toxic by-products, being prevented by a reduction of its serum levels with possible implications for the treatment [6]. The purpose of this study was to evaluate the dietary habits and nutritional intake of vitamin A in patients with STGD and RP, in order to suggest tailored dietary modifications in such diseases

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