Effect of docosahexaenoic acid supplementation on retinal function in a patient with autosomal dominant Stargardt-like retinal dystrophy (original) (raw)
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Investigative ophthalmology & visual science, 2015
Docosahexaenoic acid (DHA) was supplemented in a single-site, placebo-controlled, randomized clinical trial designed to slow vision loss associated with X-linked retinitis pigmentosa (XLRP); the DHAX Trial. We previously reported no significant differences between supplemented and placebo groups in intent-to-treat analysis of primary ERG outcomes. Assessed herein are hypothesis-generating measures of ancillary visual function outcomes in participants fully adhering to trial protocol. Male participants with XLRP (range, 7-31 years) received 30 mg DHA/kg/d (n = 29) or placebo (n = 22) for 4 years. Visual outcomes were measured annually and red blood cell (RBC) DHA determined every 6 months. Oral DHA supplementation increased mean RBC-DHA levels by 4-fold (P < 0.0001) over placebo. No group differences in progression were found for visual acuity (P = 0.11), shape discrimination (P = 0.18), or fundus appearance (P = 0.70). Optical coherence tomography (OCT) became available during ye...
Early recognition, evaluation and treatment may prevent blindness in giant cell arteritis
Expert Review of Ophthalmology, 2007
Giant cell arteritis (GCA) is a systemic vasculitis that can cause blindness in the elderly. GCA is a medical emergency since it frequently causes blindness if left untreated. Blindness can even occur in a small percentage of patients who undergo treatment. The etiology remains unknown. Presenting symptoms of GCA vary widely and visual manifestations are an extremely common mode of presentation. All physicians must have a high level of suspicion to diagnose and initiate early and aggressive treatment, which in most patients can arrest but rarely reverses the disease. Early and prolonged high-dose corticosteroids remain the cornerstone of GCA treatment. In symptomatic patients, the combination of abnormal levels of C-reactive protein in addition to Westergren erythrocyte sedimentation rate has 97% specificity in the laboratory diagnosis of GCA. The gold standard for diagnosis is the temporal artery biopsy, but biopsy-negative GCA cases have been reported.
Visual performance in giant cell arteritis (temporal arteritis) after 1 year of therapy
British Journal of Ophthalmology, 1999
Aims-To determine if patients with giant cell arteritis (GCA) treated with corticosteroids develop delayed visual loss or drug related ocular complications. Methods-In a multicentre prospective study patients with GCA (using precise diagnostic criteria) had ophthalmic evaluations at predetermined intervals up to 1 year. The dose of corticosteroid was determined by treating physicians, often outside the study, with the daily dose reduced to the equivalent of 30-40 mg of prednisone within 5 weeks. Subsequently, treatment guidelines suggested that the dose be reduced as tolerated or the patient was withdrawn from steroids in a period not less than 6 months. Results-At presentation, of the 22 patients enrolled, seven patients had nine eyes with ischaemic injury. Four eyes had improved visual acuity by two lines or more within 1 month of starting corticosteroids. No patients developed late visual loss as the steroid dose was reduced. At 1 year the visual acuity, contrast sensitivity, colour vision, and threshold perimetry were not significantly diVerent from the 4-5 week determinations. At 1 year, there were no significant cataractous or glaucomatous changes. At 2 months, there was no diVerence in systemic complications between patients who received conventional dose (60-80 mg per day) or very high doses (200-1000 mg per day) of corticosteroids at the start or early in the course.
Medicine, 2016
To determine the incidence of permanent visual loss (PVL) in giant cell arteritis (GCA) and the GCA relapse rate during glucocorticoid (GC) tapering.This prospective, longitudinal single secondary/tertiary rheumatology centre study was conducted between September 2011 and September 2014 in Slovenia. Predetermined clinical and laboratory tests were performed at 12, 24, 48, 96, and 144 weeks after diagnosis.Sixty-eight GCA patients (72.1% female), with a median (IQR) age of 73.2 (67.3-76.1) years and a symptom duration before the diagnosis of a median (IQR) 30 (14-70) days were included. Thirty-nine of 68 patients had symptoms for less than 31 days (14 (10-28) days-early GCA) and 29/68 for 31 days or longer (90 (60-120) days-late GCA). Four (5.9%) patients presented with PVL (1 early GCA). The median (IQR) follow-up was (IQR) 104 (53-126) weeks. GCA relapsed in 17/39 (43.6%) and 14/29 (48.3%) in early and late GCA, respectively. The median (IQR) time to the first relapse was 24.8 (13....
N RÉ SUMÉ Objective: Best disease is a slowly progressive macular dystrophy that typically has an onset in early childhood. Multiple lines of evidence suggest that dietary docosahexaenoic acid (DHA) protects against the development of macular degeneration. Our trial tested the effect of an oral supplement of DHA on visual function in patients with Best disease. Design: Double-masked, randomized, placebo-controlled, crossover clinical trial. Participants: Eight patients with Best disease. Methods: Patients were given either an oral supplement of DHA (20 mg/kg daily) or placebo. Primary outcome measures were the multifocal electroretinogram (mfERG) and electro-oculogram (EOG). Plasma DHA was tracked along with visual acuity (Early Treatment Diabetic Retinopathy Study chart), VF-14 scores, and Humphrey visual fields. Results: All 8 patients had increased plasma DHA levels (2-3 fold) during periods of DHA supplementation compared with periods without supplementation. Differences in visual acuity, VF-14 scores, and EOG Arden ratios during periods with and without DHA supplementation were all statistically insignificant. A positive correlation was found between the plasma concentration of DHA and mfERG amplitudes, but amplitude changes during the treatment periods were not significant. A carryover effect of DHA supplementation was a confounding error. Conclusions: Our pilot trial of DHA supplementation in 8 patients with Best disease did not demonstrate an improvement in macular function. An expanded trial would be needed to examine the full effects of DHA supplementation on visual function in Best disease.
Features and risk factors for new (secondary) permanent visual involvement in giant cell arteritis
Clinical and Experimental Rheumatology
Objective New permanent visual loss (PVL) in treated patients with giant cell arteritis (GCA) is a rare but worrisome occurrence. In this study, we aimed to describe the frequency and main features of new PVL occurring after the beginning of glucocorticoid therapy in patients with newly diagnosed GCA. Methods We included in an inception cohort all consecutive patients newly diagnosed with GCA in the internal medicine department of a tertiary-care hospital between 1976 and May 2020. The study population comprised all the patients without bilateral PVL before treatment who were followed for at least one year. Only well-documented visual events that set after the initiation of glucocorticoid treatment were regarded as new PVL. Results Eleven out of 502 patients (2.2%) experienced a new PVL including 6 occurrences during the initial therapeutic phase and 5 during the tapering phase. Patients with new PVL during treatment had higher mean age, more often displayed temporal artery abnormalities on physical examination, and had higher mean platelet counts at GCA onset. There was a strong excess risk of contralateral recurrence during treatment in patients with unilateral loss at GCA onset compared with patients with uncomplicated GCA (10.5% vs 1.1%, OR=10.26, p<0.001). Conclusion New PVL in treated GCA is a rare, but significant occurrence. Older patients and patients who already had unilateral PVL at diagnosis have higher risk of new ischaemic visual loss during treatment compared to the other patients. Close clinical, laboratory, and eye monitoring of these high-risk patients is of paramount importance.
Update on giant cell arteritis
Current Opinion in Ophthalmology, 2003
Purpose of review Giant cell arteritis (GCA) is the most common form of systemic vasculitis that causes visual loss in the elderly. This review highlights current concepts dealing with the diagnosis, treatment, and visual prognosis of patients with GCA. Recent findings Recent evidence suggests that recovery of visual function in patients with visual loss from GCA is poor. An algorithm has been constructed to assist clinicians in the evaluation and management of patients suspected of having GCA. Summary Despite a number of new adjunctive agents, corticosteroids remain the standard treatment in patients with GCA.