Fructus lycii: A Natural Dietary Supplement for Amelioration of Retinal Diseases.

Fructus lycii (F. lycii) is an exotic "berry-type" fruit of the plant Lycium barbarum that is characterized by a complex mixture of bioactive compounds distinguished by their high antioxidant potential. F. lycii is used in traditional Chinese home cooking and in the Chinese Pharmacopeia as an aid to vision and longevity as well as a remedy for diabetes to balance "yin" and "yang" in the body for about two centuries. Although a myriad of bioactive compounds have been isolated from F. lycii, polysaccharides, carotenoids, flavonoids, and phenolics represent the key functional components of F. lycii. F. lycii has been shown to exhibit a wide range of biological activities in experimental settings including antioxidant, anti-inflammatory, antiapoptotic, and neuroprotective effects. Despite its medicinal role dating back to the eighteenth century in the Far East and robust evidence of beneficial effects on ocular health and retinal diseases originating mainly from studies in animal models, the role of F. lycii in the clinical management of retinal diseases is yet to be established. This article comprehensively reviews the literature germane to F. lycii and retinal diseases with particular emphasis on age-related macular degeneration, diabetic retinopathy, and retinitis pigmentosa, which are commonly seen in clinical practice.

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

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.

An unusual ophthalmic finding in Lane-Hamilton syndrome.

Lane-Hamilton syndrome is a rare condition that is characterized by idiopathic pulmonary hemosiderosis and celiac disease. We report the case of an 18-month-old girl with Lane-Hamilton syndrome who had unilateral pigmentary retinopathy.

Antioxidants and vision health: facts and fiction.

A number of nutritional supplements containing antioxidants are advertised for better vision health. Do they benefit the average consumer? The literature was examined for the effectiveness of antioxidants for human eye health, and for the intricacies in collection of such evidence. The following diseases were considered: cataract, glaucoma, age-related macular degeneration (AMD), retinopathy, retinitis pigmentosa, eye infections, and uveitis. The literature indicates that antioxidant supplements plus lutein have a reasonable probability of retarding AMD. For glaucoma, such supplements were ineffectual in some studies but useful in others. In some studies, antioxidant rich fruits and vegetables were also useful for protection against glaucoma. For diabetic retinopathy, antioxidant supplements may have a small benefit, if any, but only as an adjunct to glycemic control. In very high-risk premature retinopathy and retinitis pigmentosa, antioxidant supplements may be beneficial but those with excess Vitamin E should be avoided. For cataract, there is no evidence for an advantage of such nutritional supplements. However, lubricant drops containing N-acetylcarnosine may be helpful in initial stages of the disease. For eye infections and other causes of uveitis, antioxidants have not been found useful. We recommend that a diet high in antioxidant rich foods should be developed as a habit from an early age. However, when initial signs of vision health deterioration are observed, the appropriate nutritional supplement products may be recommended but only to augment the primary medical treatments.

ω-3 intake and visual acuity in patients with retinitis pigmentosa receiving vitamin A.

OBJECTIVE: To evaluate whether a diet high in long chain ω-3 fatty acids can slow the rate of visual acuity loss among patients with retinitis pigmentosa receiving vitamin A palmitate. METHODS: We calculated dietary intake from questionnaires completed annually by 357 adult patients from 3 randomized trials who were all receiving vitamin A, 15 000 IU/d, for 4 to 6 years. Rates of visual acuity decline were compared between those with high (≥0.20 g/d) vs low (<0.20 g/d) ω-3 intake. Analyses took age into account. RESULTS: Mean rates of decline of acuity were slower among those with high ω-3 intake: Early Treatment Diabetic Retinopathy Study distance acuity: high intake=0.59 letter per year, low intake=1.00 letter per year,P=.001; Snellen retinal acuity: high intake=1.5% per year, low intake=2.8% per year, P=.03. CONCLUSIONS: We conclude that mean annual rates of decline in distance and retinal visual acuities in adults with retinitis pigmentosa receiving vitamin A, 15 000 IU/d,are slower over 4 to 6 years among those consuming a diet rich in ω-3 fatty acids. To our knowledge, this is the first report that nutritional intake can modify the rate of decline of visual acuity in retinitis pigmentosa.

Disc swelling in abetalipoproteinemia: a novel feature of Bassen-Kornzweig syndrome.

PURPOSE: Abetalipoproteinemia is a very rare disease with multisystemic manifestations. METHODS: Retrospective, case report. RESULTS: We report a 9-year-old girl with a history of Bassen-Kornzweig syndrome diagnosed at the age of 3. The patient was referred to us by the pediatricians for ocular assessment. During the examination, an atypical pigmentary retinopathy and bilateral swelling of the optic discs were observed, with no consequences to her visual acuity. CONCLUSIONS: Ophthalmic manifestations in Bassen-Kornzweig syndrome are not uncommon. We describe, for the first time, bilateral swelling of the optic discs, a sign that appears directly associated with abetalipoproteinemia.

Unusual presentation: pulmonary hemosiderosis with celiac disease and retinitis pigmentosa in a child.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9-year-old boy with Lane-Hamilton syndrome, co-occurrence of pulmonary hemosiderosis with CD. This presentation is unique presentation because he has also retinal pigmentation.

[Diet and management of degenerative diseases of the retina (retinitis pigmentosa)]. / Dieta e Vitamina A nel trattamento di forme "sensibili" di retinite pigmentosa.

Considerable progress has been made in the understanding and management of degenerative diseases of the retina. The dietetic intervention has been favourably proposed in the most common forms of retinitis pigmentosa, a condition potentially leading to blindness. Vitamin A has been shown to be effective in delaying progression of the disease. In these patients such treatment is the only possible therapy, to date, and a lifetime generous supplementation of retinol is advisable, together with a vitamin A-rich diet and/or a dietary supplement (e.g. carrot flour) or pharmacologic supplement of vitamin A. Supply of vitamin A in doses up to 25000 IU (7500 igr/day), even for several years, has so far proved safe from risk of occurrence of liver disease. A possible effect on hypercholesterolemia related to a very prolonged treatment in predisposed individuals can be avoided by using a special diet, particularly enriched with beta-carotene. Guidelines for preparing a diet, specially formulated to provide an elevated weekly supply of vitamin and/or its precursor (equal to 15000 IU or 5000 microg of RE, retinol equivalent) and to control possible risk factors related to dietetic manipulation (supply of fat lower than 30% of total calories, variable levels of cholesterol and polyunsaturated fatty acids n-3, n-6) are presented. As long as resolutive therapy is lacking, dietetic intervention plays a primary role, although underestimated, in the management of the patients suffering from retinitis pigmentosa. The diet is specifically characterized by presence of food with a high content of carotenoids, substances with a favourable and additive effect.

Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.

PURPOSE: To define the ophthalmologic findings in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, an inborn error of mitochondrial beta-oxidation. METHOD: Case report. RESULTS: A 5-year-old girl with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency had a bilateral acquired disturbance of the retinal pigment epithelium consisting of a central macular spot and regularly spaced peripheral spots. Central and peripheral vision and dark adaptation appeared to be mildly compromised. Electroretinography showed abnormalities of the cone system. CONCLUSIONS: An excess of long chain and very long chain fatty acid intermediates has been postulated as the cause of the retinopathy in long chain 3-hydroxyacyl-CoA dehydrogenase deficiency and the biochemically related peroxisomal disorders. Dietary management may slow or halt progression. Ophthalmoscopic detection of regularly spaced pigment spots could help identify long chain 3-hydroxyacyl-CoA dehydrogenase deficiency in future cases.

[Refsum syndrome in a pair of monozygotic twins]. / Refsum-Syndrom bei einem eineiigen Zwillingspaar.

We report the case of a pair of twins with the ophthalmological and functional findings of Refsum syndrome. The twins were monozygotic twin brothers whose ophthalmological symptoms were noticed when they were in their forties. The diagnosis in the first brother led to a search for findings in the second. It was possible to attribute the non-specific subjective complaints to this as yet unknown syndrome. No causal therapy can be offered the patients. However, knowledge of the biochemical basis of the disease makes it possible to treat it by means of diet. In patients with retinopathia pigmentosa, an attempt should be made to identify Refsum syndrome if neurological symptoms are found in addition.