Dietary profile of patients with Stargardt’s disease and Retinitis Pigmentosa: is there a role for a nutritional approach?
Open accessAbstract
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.
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Treatment with vitamin A in retinal diseases associated with vitamin A is not straight-forward because of fear of accumulation of excessive amounts of toxic retinoids formed as byproducts of vitamin A pathways.
“Treatment with vitamin A in these cases is not straight-forward, as there is a fear of accumulation of excessive amounts of toxic retinoids that are formed as byproducts of vitamin A pathways.”
From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [76]
The Discussion says that impaired vitamin A metabolism in Stargardt’s disease produces toxic byproducts, and the Background reports that supplementation accelerated their accumulation in an animal model. But the paper distinguishes retinitis pigmentosa, for which it reports possible benefits from vitamin A supplementation; it does not present toxic-byproduct accumulation as the concern across both diseases. The supplied passages have section labels but no page numbers. (LLM, conf=0.84)
Experimental — accuracy not yet measuredIn ABCA4-retinopathy, toxic products of vitamin A accumulate excessively in the retina.
“A good example of this toxicity is ABCA4-retinopathy where toxic products of vitamin A accumulate excessively in the retina [9,23,76,77,78].”
From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [76]
High vitamin A intake is a risk factor for progression of ABCA4-retinopathy.
“According to dietary questionnaire studies and animal model studies, high vitamin A intake is thought to be a risk factor for the progression [76,98], therefore patients are discouraged from taking vitamin A supplements.”
From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [76]
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- Authors
- Sofi, F., Sodi, A., Franco, F., Murro, V., Biagini, D., Miele, A., Abbruzzese, G., Mucciolo, D. P., Virgili, G., Menchini, U., Casini, A., & Rizzo, S.
- Affiliations
- Azienda Ospedaliero-Universitaria Careggi (IT)
- University of Florence (IT)
- Don Carlo Gnocchi Foundation (IT)
- Corresponding author
- Sodi, Andrea
- ORCID
- Venue
- BMC Ophthalmology
- Published
- 2016-01-22
- Volume
- 16
- Issue
- 1
- Pages
- 13-13
- ISSN
- 1471-2415 (ISSN-L)
- PMC
- PMC4722760
- OpenAlex
- W2253450442
- Cited by
- 24
- References
- 20
- Citations by year
- 2026: 5 · 2025: 1 · 2024: 1 · 2023: 2 · 2022: 6 · 2020: 4 · 2019: 2 · 2018: 3
- FWCI
- 0.87
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- BMC Ophthalmology (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Florence Research (University of Florence) (submittedVersion, other-oa)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinal Development and Disorders
- Health Sciences › Medicine › Ophthalmology › Retinal Diseases and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinoids in leukemia and cellular processes
- Keywords
- Stargardt disease, retinitis pigmentosa, inherited retinal degeneration, dietary intake, vitamin A, dietary intervention, visual acuity, macular degeneration
- MeSH terms
- Stargardt Disease, Adolescent, Adult, Aged, Aged, 80 and over, Energy Intake, Diet, Diet Surveys, Feeding Behavior, Female, Humans, Macular Degeneration, Male, Middle Aged, Retinitis Pigmentosa, Visual Acuity, Vitamin A, Nutrition Assessment, Young Adult
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