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Open Access 01-12-2016 | Research article

Dietary profile of patients with Stargardt’s disease and Retinitis Pigmentosa: is there a role for a nutritional approach?

Authors: 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

Published in: BMC Ophthalmology | Issue 1/2016

Abstract

Background

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

Methods

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.

Results

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.

Conclusion

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.

Lien EI, Hammond BR. Nutritional influences on visual development and function. Progr Ret Eye Res. 2011;30:188–203.CrossRef

Palczewski K. Chemistry and Biology of Vision. J Biol Chem. 2012;287:1612–9.CrossRefPubMed

Chappelow AV, Traboulsi EI. Stargardt Disease. In: Traboulsi EI, editor. Genetic Diseases of the Eye. Oxford-New York: Oxford University Press; 2012. p. 467–75.CrossRef

Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet. 2006;368:1795–809.CrossRefPubMed

Ferreyra HA, Heckenlively JR. Retinitis Pigmentosa. In: Traboulsi EI, editor. Genetic Diseases of the Eye. Oxford-New York: Oxford University Press; 2012. p. 381–92.CrossRef

Radu RA, Yuan Q, Hu J, Peng JH, Lloyd M, Nusinowitz S, et al. Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation. Invest Ophthalmol Vis Sci. 2008;49:3821–9.CrossRefPubMedPubMedCentral

Aleman TS, Cideciyan AV, Windsor EA, Schwartz SB, Swider M, Chico JD, et al. Macular pigment and lutein supplementation in ABCA4-associated retinal degenerations. Invest Ophthalmol Vis Sci. 2007;48:1319–29.CrossRefPubMedPubMedCentral

Berson EL, Rosner B, Sandberg MA, Hayes KC, Nicholson BW, Weigel-DiFranco C, et al. A randomized trial of vitamin A and vitamin E supplementation for retinitis pigmentosa. Arch Ophthalmol. 1993;111:761–72.CrossRefPubMed

Berson EL, Rosner B, Sandberg MA, Moser A, Brockhurst RJ, Hayes KC, et al. Clinical trial of docosohexanoic acid in patients with Retinitis Pigmentosa receiving vitamin A treatment. Arch Ophthalmol. 2004;122:1297–305.CrossRefPubMed

10.

Berson EL, Rosner B, Sandberg MA, Weigel-DiFranco C, Moser A, Brockhurst RJ, et al. Further evaluation of docosohexanoic acid in patients with Retinitis Pigmentosa receiving vitamin A treatment. Arch Ophthalmol. 2004;122:1306–14.CrossRefPubMed

11.

Berson EL, Rosner B, Sandberg MA, Weigel-Di Franco C, Willett W. ω-3 intake and visual acuity in patients with Retinitis Pigmentosa receiving vitamin A. Arch Ophthalmol. 2012;130:707–11.CrossRefPubMedPubMedCentral

12.

Berson EL, Rosner B, Sandberg MA, Weigel-DiFranco C, Brockhurst RJ, Hayes KC, et al. Clinical trial of lutein in patients with Retinitis Pigmentosa receiving vitamin A. Arch Ophthalmol. 2010;128:403–11.CrossRefPubMedPubMedCentral

13.

Koenekoop RK. The gene for Stargardt disease, ABCA4, is a major retinal gene: a mini-review. Ophthalmic Genet. 2003;24:75–80.CrossRefPubMed

14.

Passerini I, Sodi A, Giambene B, Mariottini A, Menchini U, Torricelli F. Novel mutations of the ABCR gene in Italian patients with Stargardt Disease. Eye. 2010;24:158–64.CrossRefPubMed

15.

Italian Society of Human Nutrition. Livelli di Assunzione di Riferimento di Nutrienti ed energia per la popolazione italiana 2012. Available at: http://www.sinu.it/html/pag/larn-2014.asp (accessed on August, 28 2013).

16.

Perusek L, Maeda T. Vitamin A derivatives as treatment options for retinal degenerative diseases. Nutrients. 2013;5:2646–66.CrossRefPubMedPubMedCentral

17.

Radu RA, Han Y, Bui TV, Nusinowitz S, Bok D, Lichter J, et al. Reductions in serum vitamin A arrest accumulation of toxic retinal fluorophores: a potential therapy for treatment of lipofuscin-based retinal diseases. Invest Ophthalmol Vis Sci. 2005;46:4393–401.CrossRefPubMed

18.

Massof RW, Fishman GA. How strong is the evidence that nutritional supplements slow the progression of Retinitis Pigmentosa? Arch Ophthalmol. 2010;128:493–5.CrossRefPubMed

Title: Dietary profile of patients with Stargardt’s disease and Retinitis Pigmentosa: is there a role for a nutritional approach?
Authors: 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
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: BMC Ophthalmology / Issue 1/2016
Electronic ISSN: 1471-2415
DOI: https://doi.org/10.1186/s12886-016-0187-3

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Dietary profile of patients with Stargardt’s disease and Retinitis Pigmentosa: is there a role for a nutritional approach?

Abstract

Background

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

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