Dietary profile of patients with Stargardt's disease and Retinitis Pigmentosa: is there a role for a nutritional approach?
Francesco Sofi,Andrea Sodi,Fabrizio Franco,Vittoria Murro,Dania Biagini,Alba Miele,Giacomo Abbruzzese,Dario Pasquale Mucciolo,Gianni Virgili,Ugo Menchini,Alessandro Casini,Stanislao Rizzo +11 more
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TLDR
Interestingly, STGD patients with low vitamin A intake showed significantly better visual acuity with respect to those introducing higher intake of vitamin A, especially for daily intake of Vitamin A.Abstract:
Stargardt’s disease (STGD) and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite way, by diet. Dietary 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. Interestingly, 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. The 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.read more
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The Impact of Inherited Retinal Diseases in the Republic of Ireland (ROI) and the United Kingdom (UK) from a Cost-of-Illness Perspective.
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Highly Variable Disease Courses in Siblings with Stargardt Disease.
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References
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A randomized trial of vitamin A and vitamin E supplementation for retinitis pigmentosa.
Eliot L. Berson,Bernard Rosner,Michael A. Sandberg,Kenneth C. Hayes,Britain W. Nicholson,Carol Weigel-DiFranco,Walter C. Willett +6 more
TL;DR: These results support a beneficial effect of 15 000 IU/d of vitamin A and suggest an adverse effect of 400 IU/D of vitamin E on the course of retinitis pigmentosa.
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Chemistry and Biology of Vision
TL;DR: This work has shown that light-activated signaling in the retina is initiated by the 11-cis-retinal chromophore complex, a vitamin A derivative bound to rhodopsin and cone opsins of retinal photoreceptors, which is a nonconventional retinal opsins based on similar photochemistry.
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Reductions in serum vitamin A arrest accumulation of toxic retinal fluorophores: a potential therapy for treatment of lipofuscin-based retinal diseases.
Roxana A. Radu,Yun Han,T. V. Bui,Steven Nusinowitz,Dean Bok,Jay Lichter,Widder Kenneth,Gabriel H. Travis,Nathan L. Mata +8 more
TL;DR: These findings demonstrate the vitamin A-dependent nature of A2E biosynthesis and validate a novel therapeutic approach with potential to halt the accumulation of lipofuscin fluorophores in the eye.
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Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation.
Roxana A. Radu,Quan Yuan,Jane Hu,J. H. Peng,Marcia Lloyd,Steven Nusinowitz,Dean Bok,Gabriel H. Travis +7 more
TL;DR: Vitamin A supplementation should be avoided in patients with ABCA4 mutations or other retinal or macular dystrophies associated with lipofuscin accumulation in the retinal pigment epithelium.