Why Miss the Chance? Incidental Findings while Telescreening for Diabetic Retinopathy.

PURPOSE: To report on incidental pathological findings met while screening for Diabetic Retinopathy (DR) in Diabetes Clinics (DC) by ophthalmologist-graded digital fundus imaging. METHODS: At the DC of Pescara (central Italy), for 3,859 eyes of 1,930 consecutive patients having not undergone fundus examination in the last year, two mydriatic fundus digital images, taken with a CenterVue DRS Digital Retinal Camera, were sent along with Best Corrected Visual Acuity, on a "store-and-forward" basis, to an ophthalmologist trained in DR screening, and graded according to the UK Diabetic Eye Screening Programme. Incidental fundus abnormalities other than DR were reported. RESULTS: No adverse event to mydriasis was reported. One hundred and eighty eyes (4.66%) were ungradable. Among the 3,679 gradable ones, 1,105 (30.04%) showed different degrees of DR (R1 to R3), and 126 (3.42%) maculopathy (M1). Any Age-Related Macular Degeneration was present in 387 eyes (10.52%), any optic disc and parapapillary area features suspect for glaucoma in 562 eyes (15.27%), any hypertensive retinopathy in 1,263 eyes (34.33%), vitreoretinal interface disease in 252 eyes (6.84%), myopic choroidopathy in 92 eyes (2.50%), disc pallor in 31 eyes (0.84%). Mean time was 5 min for screening, 2 min for grading. CONCLUSION: Teleretinography is a well-established, cost-effective procedure in DR screening. Along with increased attendance, locating a digital camera in a DC with a retina-specialist grader results in finding fundus pathologies also beyond DR, very similarly to fundus examination in an outpatient ophthalmic setting.

Coexistence of Peripheral Retinal Diseases with Macular Hole

Objectives: To investigate the frequency of retinal tear, retinal hole, and lattice degeneration in peripheral retinal examination of patients with macular hole. Materials and Methods: The files of patients who underwent pars plana vitrectomy surgery with a diagnosis of macular hole at Eskisehir Osmangazi University Department of Ophthalmology between 2008 and 2018 were retrospectively analyzed. A total of 106 patients with primary macular hole who underwent peripheral retinal examination were included in the study. The frequency of retinal tears, holes, and lattice degeneration associated with macular hole was investigated. Results: Peripheral retinal examination of 106 patients who underwent macular hole surgery revealed retinal tear in 3 patients (2.8%), retinal hole in 4 patients (3.8%), and lattice degeneration in 10 patients (9.4%). Retinal hole and lattice degeneration were observed concomitantly in 1 patient. Conclusion: This study showed that patients with macular hole have concomitant retinal tears and holes, which are also thought to arise due to vitreoretinal traction, at a frequency similar to that in the general population. This result suggests that both the anterior and posterior vitreous may have different pathologies at the same time related to these diseases.

Optical coherence tomography and molecular analysis of sudden acquired retinal degeneration syndrome (SARDS) eyes suggests the immune-mediated nature of retinal damage.

OBJECTIVE: To perform detailed analysis of retinal changes in dogs with SARDS using optical coherence tomography (OCT), funduscopy, and molecular analysis. ANIMALS: Subjects were 29 dogs from 12 US states and Canada diagnosed with SARDS by 8 ophthalmologists. An additional 7 eyes from 5 deceased SARDS dogs were used for molecular and histological analysis. PROCEDURES: Dogs were evaluated using chromatic pupil light reflex testing (cPLR), and electroretinography (ERG); subjects underwent complete ophthalmic examination, including funduscopy, retinal photography, and OCT, in addition to complete laboratory analysis, blood pressure evaluation, abdominal and thoracic radiographs, and computerized tomography (CT) imaging to assess possible systemic abnormalities. Histology and immunohistochemistry analysis was performed in 2 SARDS eyes. Microarray analysis was performed in 5 SARDS retinas. RESULTS: Thirty-eight percent of patients had <1-mm wide retinal detachments (RD) on OCT analysis, which could not be detected by funduscopy or retinal photographs. Systemic hypertension did not seem to be a contributing factor (RD 22.2%; ND 20%, Odds ratio = 1.1). No dogs showed neoplastic changes by thoracic or abdominal radiography, or CT imaging. There was no statistically significant difference in age (RD 7.9 ± 1.9 years (mean ± SD); ND 7.6 ± 1.7 years, p = 0.69) or duration of blindness prior to presentation (RD 18 ± 7 days (mean±SD); ND 21 ± 12 days, p = 0.28). Microarray and histology analysis of SARDS eyes revealed molecular changes suggestive of immune-mediated damage. CONCLUSIONS: Observed histological, molecular, and OCT changes are highly suggestive of immune-mediated damage in SARDS eyes.

Gene Therapy for Retinal Degeneration.

Biallelic mutations in the RPE65 gene are associated with inherited retinal degenerations/dystrophies (IRD) and disrupt the visual cycle, leading to loss of vision. A new adenoviral vector-based gene therapy surgically delivered to retinal cells provides normal human RPE65 protein that can restore the visual cycle and some vision. To view this Bench to Bedside, open or download the PDF.

Sudden acquired retinal degeneration syndrome in western Canada: 93 cases.

This study reviewed clinical data from dogs diagnosed with sudden acquired retinal degeneration syndrome (SARDS) in western Canada. Medical records from the Western College of Veterinary Medicine from 2002 to 2016 showed that 93 cases of SARDS were diagnosed based on presentation for sudden blindness and a bilaterally extinguished electroretinogram. The most common pure breeds were the miniature schnauzer, dachshund, and pug. The mean age at diagnosis was 8.1 years and males and females were equally affected. Most of the dogs were presented with normal non-chromatic, but abnormal chromatic pupillary light reflexes. The incidence of retinal degeneration as detected via ophthalmoscopy increased over time after SARDS diagnosis. Polyuria, polydipsia, polyphagia, weight gain, elevated liver enzyme values, isosthenuria, and proteinuria were common clinical and laboratory findings. Chromatic pupillary light reflex testing may be more valuable than non-chromatic pupillary light testing in detecting pupil response abnormalities in dogs with SARDS, although electroretinography remains the definitive diagnostic test.

Syndrome de la rétine silencieuse dans l'Ouest canadien : 93 cas. Cette étude a examiné les données cliniques provenant de chiens diagnostiqués avec le syndrome de la rétine silencieuse (syndrome de cécité soudaine acquise) dans l'Ouest canadien. Les dossiers médicaux du Western College of Veterinary Medicine de 2002 à 2016 ont montré que 93 cas du syndrome de la rétine silencieuse ont été diagnostiqués en se basant sur la présentation pour une cécité soudaine et un électrorétinogramme bilatéral sans incandescence. Les races les plus communes étaient le Schnauzer miniature, le Dachshund et le Pug. L'âge moyen au diagnostic était de 8,1 ans et les mâles et les femelles étaient également affectés. La plupart des chiens présentaient des réflexes pupillaires normaux à la lumière non chromatique mais des réflexes anormaux à la lumière chromatique. L'incidence de la dégénération rétinienne détectée par l'ophtalmoscopie a augmenté au fil du temps après le diagnostic du syndrome de la rétine silencieuse. La polyurie, la polydipsie, la polyphagie, le gain de poids, des valeurs d'enzymes hépatiques élevées, l'isosthénurie et la protéinurie étaient des résultats cliniques et de laboratoire communs. Le réflexe à la lumière pupillaire chromatique peut être plus utile que le test de la lumière pupillaire non chromatique pour détecter les anomalies de la réponse pupillaire chez les chiens atteints du syndrome de la rétine silencieuse, quoique l'électrorétinographie demeure le test diagnostique définitif.(Traduit par Isabelle Vallières).

The incidence and risk factors for the development of vitreomacular interface abnormality in diabetic macular edema treated with intravitreal injection of anti-VEGF.

PurposeTo report the incidence and associated factors for the development of vitreomacular interface abnormality (VMIA) in patients with diabetic macular edema (DME) who received intravitreal injection (IVI) of anti-VEGF (Bevacizumab and Ranibizumab) treatment.MethodsA retrospective observational study. Patients with DME followed at least 6 months were reviewed. Baseline best-corrected visual acuity (BCVA), central retinal thickness (CRT) and final BCVA, CRT in eyes with and without VMIA were compared. Multiple logistic regression was also used to investigate the risk factors of VMIA formation in patients with DME treated by anti-VEGF.ResultsA total of 201 eyes in 142 patients met the inclusion criteria of the study. VMIA developed in 44 eyes (21.89%) of patients during a mean follow-up period of 40.84 months. The estimated mean incidence of VMIA formation was 6.43% per year. Poor baseline BCVA was found to be a risk factor for VMIA development (P=0.001, odds ratio=5.299, 95% confidence interval: 1.972 to 14.238). There was no difference between eyes with and without VMIA formation in improving BCVA (P=0.557) and lowering the macular edema (eyes without VMIA formation: -107.72±171.91 µm; eyes with VMIA formation: -155.02±212.27 µm, P=0.133).ConclusionsThis study revealed the incidence of VMIA formation in IVI anti-VEGF treated DME eyes was 6.43%. Poor baseline BCVA was found to be a risk factor for VMIA formation. Both eyes with and without VMIA development had favorable response to anti-VEGF treatment.

PERIPHERAL RETINAL DEGENERATIONS AND RHEGMATOGENOUS DETACHMENT IN PRIMARY CONGENITAL GLAUCOMA.

BACKGROUND: To determine the prevalence of peripheral retinal degenerations (PRD) and rhegmatogenous retinal detachment in patients with primary congenital glaucoma (PCG). METHODS: Records of all patients with PCG operated from year 2000 onwards were evaluated to look for the prevalence of rhegmatogenous retinal detachment. Of these, those children who were old enough to cooperate and had sufficient medial clarity were screened with an indirect ophthalmoscopy in a cross-sectional evaluation from 2010 to 2014. Peripheral retina was examined, and prevalence of PRD was estimated in this subset. For statistical purposes, only one eye of each patient was considered in this cross-sectional analysis. RESULTS: Of the 310 eyes (180 patients with PCG) operated from the year 2000 onwards, a rhegmatogenous retinal detachment was noted in 13 eyes (4%). Mean axial length of these eyes was 26.3 ± 3.2 mm (range, 19.8-34.7 mm). Among the eyes screened for PRD (n = 60), prevalence of pathologic PRD (lattices with/without atrophic holes and isolated holes/tears) was 15%. The average follow-up between glaucoma filtering surgery and the date of last examination was 8.55 ± 3.98 years (range, 5-20 years) in this subset. Mean axial length was significantly greater in eyes with pathologic PRD than in those without (28.1 ± 3.3 mm vs. 25.8 ± 2.6 mm; P = 0.01). For axial length ≥ 26 mm, the odds of having a pathologic PRD were 14.4 times more than those with axial length < 26 mm (P < 0.001; 95% confidence interval, 1.7-120.5). CONCLUSION: Prevalence of PRD among eyes with PCG is high. Peripheral retinal screening should be performed in eyes with PCG, especially those with axial lengths ≥ 26 mm.

Therapy strategies for Usher syndrome Type 1C in the retina.

The Usher syndrome (USH) is the most common form of inherited deaf-blindness with a prevalence of ~ 1/6,000. Three clinical subtypes (USH1-USH3) are defined according to the severity of the hearing impairment, the presence or absence of vestibular dysfunction and the age of onset of retinitis pigmentosa (RP). USH1 is the most severe subtype with congenital severe to profound hearing loss and onset of RP before puberty. Currently only the amelioration of the hearing deficiency is implemented, but no treatment of the senso-neuronal degeneration in the eye exists.In our studies we are focusing on the evaluation of gene-based therapies to cure the retinal degeneration of USH1C patients: (i) gene augmentation using recombinant adeno-associated virus, (ii) genome editing by homologous recombination mediated by zinc-finger nucleases and, (iii) read-through therapy using novel designer aminoglycosides and PTC124. Latter compounds target in-frame nonsense mutations which account for ~ 20 % of all USH cases.All analyzed gene-based therapy strategies lead to the restoration of USH protein expression. These adjustments may be sufficient to reduce the progression of retinal degeneration, which would greatly improve the life quality of USH patients.

Characteristics and pattern of rhegmatogenous retinal detachment in pakistan.

OBJECTIVE: To determine the presenting characteristics of rhegmatogenous retinal detachment (RRD) in a consecutive series of Pakistani patients. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Layton Rahmatullah Benevolent Trust (LRBT), Lahore, from June 2010 to October 2011. METHODOLOGY: A total of 107 eyes underwent RRD surgery. Detailed diagrams were drawn pre-operatively for all. Other recorded details included, bio-data, age, gender, visual acuity, autorefractometery, predisposing risk factors, lens state and duration of symptoms. The data was analyzed with respect to age and gender distribution, frequency of various risk factors for RRD, duration of RRD and its various presenting characteristics. RESULTS: Mean age of the patients were 33.1 ± 1.5 years, 85% were males. Bilateral RRD was discovered in 2.8% patients. No identifiable risk factor for RRD was present in 28%, while in rest of the 72% eyes, the commonest risk factors were intraocular surgery, trauma and peripheral myopic degeneration in decreasing order of frequency. There were 70.1% phakic, 23.4% pseudophakic and 6.5% aphakic eyes. Majority (93.5%) presented with macula off detachment. There was an overall average time lapse of 97.24 ± 16.95 days between presenting and first occurrence of symptoms. In 31.8% of eyes no break could be found pre-operatively, while 38.3% had 1 and 29.9% eyes had ³ 2 breaks. Majority of the primary breaks were horse-shoe tears (42.1%). Total RRD was found in 35.5% eyes. Infero-temporal and supero-temporal were the most frequently involved quadrants (93.5% and 86.9% respectively). CONCLUSION: Intraocular surgery, trauma and lattice degeneration in myopia are established common risk factors for RRD. These patients deserve meticulous fundus examination with indentation indirect ophthalmoscopy by trained professionals.

Age related macular degeneration and presence of posterior vitreous detachment.

PURPOSE: To find correlation between presence of PVD and incidence of different forms of AMD. MATERIAL AND METHODS: 210 eyes of patients with AMD were examined. Fluorescein angiography determined type of AMD and ultrasound examination evaluated presence of PVD. Control group included 164 eyes of patients routinely admitted to the hospital for cataract surgery. Inclusion criterium was lack of AMD and retinal medical history. RESULTS: In the control group prevalence of PVD was higher in women than in men--respectively 50.5% and 23.6%. PVD was more frequent in male patients with dry AMD (50%) than in male controls (23.6%). PVD was also more frequent in female patients with dry form of AMD (69.2%) than in controls (50.5%). In female group with AMD PVD was more frequent in patients with dry form of AMD than in patients with wet form of AMD (69.2% and 44.8%). In patients with dry AMD, PVD was statistically more frequent in women (69.2%) than in men (50%). Statistically significant was the difference between prevalence of PVD in dry and wet group (male and females together)--60.7% in dry AM and 42.6% ind wet AMD. CONCLUSION: Persistence of vitreal adhesion and traction with age might lead to a shift of the dry form into wet form of AMD. Detachment of the vitreous in dry AMD might secure the persistence of dry form. Women, due to early PVD are more prone to complications resulting from vitreoretinal traction.

[Causes of visual impairment in Mie Prefecture during a 5-year period].

PURPOSE: The purpose of this study was to determine the causes of visual impairment in Mie prefecture during a five-year period. SUBJECTS AND METHODS: The study was conducted between April 2004 and March 2009 in Mie Prefecture. 1,322 visually impaired people as defined by the Act on Welfare of Physically Disabled Persons were enrolled. We reviewed age, sex, causes of visual impairment, degree of disability and medical conditions according to their physical disability certificate. RESULTS: The four major causes of visual impairment were glaucoma (20.3%), diabetic retinopathy (18.9%), retinitis pigmentosa (12.2%), and macular degeneration (9.1%), followed by chorioretinal degeneration, stroke or brain tumor, optic atrophy, and cataract. The average ages of the four major causes were glaucoma (77.1 years), diabetic retinopathy (65.1 years), retinitis pigmentosa (62.5 years) and macular degeneration (77.7 years). CONCLUSIONS: The most common cause of visual impairment in Mie prefecture was glaucoma. The four major causes in Mie prefecture were the same as the results of the nation-wide investigation reported in 2006.

Estudio de las distrofias vitreorretinianas en población mexicana / Study of vitreoretinal dystrophies in a Mexican population

BACKGROUND: We undertook this study to demonstrate the incidence of vitreoretinal dystrophies in a Mexican population. METHODS: This was a retrospective, observational, descriptive, transverse study. We analyzed the files of patients treated at the Retina Department of a medical center for state employees (ISSSTE) from January 1991 to December 2006 to obtain the incidence of vitreoretinal dystrophies. RESULTS: We studied 36,300 patient files. We found an incidence of 0.008% for familial exudative vitreoretinal dystrophy, 0.008% for X-linked juvenile retinoschisis, 0.005% for Wagner disease and 0.005% for Goldmann-Favre disease. We present here a representative case of each type of dystrophy. CONCLUSIONS: Vitreoretinal dystrophies are uncommon diseases and are difficult to diagnose. Even though their incidence is low, the poor evolution to blindness requires identification of early signs in order to offer timely and opportune treatment.

Clinical and molecular genetics of Leber's congenital amaurosis: a multicenter study of Italian patients.

PURPOSE: To identify the molecular basis of Leber's congenital amaurosis (LCA) in a cohort of Italian patients and to perform genotype-phenotype analysis. METHODS: DNA samples from 95 patients with LCA were analyzed by using a microarray chip containing disease-associated sequence variants in eight LCA genes. In addition, all patients in whom no mutations were identified by microarray were subjected to sequence analysis of the CEP290 gene. Patients with mutations identified underwent a detailed ophthalmic evaluation. RESULTS: Disease-causing mutations were identified in 28% of patients, and twelve novel variants were identified. Mutations occurred more frequently in the RPE65 (8.4%), CRB1 (7.4%), and GUCY2D (5.2%) genes. Mutations in CEP290 were found in only 4.2% of the patients analyzed. Clinical assessment of patients carrying RPE65 or CRB1 mutations revealed the presence of retained visual capabilities in the first decade of life. RPE65 mutations were almost always associated with normal macular thickness, as assessed by optical coherence tomography (OCT), whereas CRB1 mutations were associated with reduced retinal thickness and a coarsely laminated retina. Fundus autofluorescence was mostly observed in patients with RPE65 and GUCY2D mutations and was not elicitable in patients carrying CRB1. CONCLUSIONS: RPE65 gene mutations represented a significant cause of LCA in the Italian population, whereas GUCY2D and CEP290 mutations had a lower frequency than that found in other reports. This finding suggests that the genetic epidemiology of LCA in Italy is different from that reported in the United States and in northern European countries. Autofluorescence in patients with RPE65 mutations was more frequently associated with preserved retinal thickness, which suggests that these mutations are not associated with progression of retinal degeneration. Therefore, normal retinal thickness (identified with OCT) and fundus autofluorescence may be the means with which to identify patients with LCA who carry RPE65 mutations, which are expected to be a potential gene therapy target in the near future.

Spectrum of pattern dystrophy in pseudoxanthoma elasticum.

OBJECTIVE: To study the prevalence, type, and features of pattern dystrophy in patients with pseudoxanthoma elasticum (PXE). METHODS: A search of the photographic records at the Vanderbilt Eye Institute using the keywords "angioid streaks and pseudoxanthoma elasticum" yielded 28 names. Of the 23 subjects meeting the patient selection criteria, 22 were confirmed to have a positive diagnosis for PXE after reviewing the medical history information. The diagnosis was confirmed by the constellation of fundus findings in all 22 subjects, by a clinical examination of the skin in 9, and by a skin biopsy specimen in 1. RESULTS: Pattern dystrophy was present in 16 patients (27 eyes) of those with PXE. Fourteen patients (23 eyes) had fundus pulverulentus, 3 patients (5 eyes) had butterfly-shaped dystrophy, and 1 patient (2 eyes) each had fundus flavimaculatus and reticular dystrophy. One eye of one patient developed solitary vitelliform pattern dystrophy during follow-up. Two patients showed progression from one pattern into another during follow-up. Another patient, who at first showed no evidence of pattern dystrophy in either eye, developed fundus pulverulentus in both eyes 5 years later. One patient had simultaneous evidence of 2 types: butterfly and fundus flavimaculatus pattern in each eye. Angioid streaks were seen in each eye of all patients. Peau d'orange was noted in 18 patients, optic nerve drusen in 5, and retinal crystalline bodies in 9. Choroidal neovascular membrane was present in 15 patients. CONCLUSIONS: All 5 varieties of pattern dystrophy, 2 of which were not previously associated with PXE, were seen in patients with PXE. Fluorescein angiogram was useful in delineating the type and extent of pattern dystrophy.

Prevalence and correlates of advanced retinopathy in a large selected hypertensive population. The Evaluation of Target Organ Damage in Hypertension (ETODH) study.

OBJECTIVE: To describe the prevalence of advanced retinal microvascular lesions and their associations with cardiac and extracardiac signs of target organ damage (TOD) in a large selected hypertensive population. METHODS: A total of 2172 non-diabetic untreated and treated uncomplicated essential hypertensives consecutively attending for the first time our hospital outpatient hypertension clinic and included in the Evaluation of Target Organ Damage in Hypertension (ETODH), an observational ongoing registry of hypertension-related TOD, were considered for this analysis. Advanced hypertensive retinopathy was defined by the presence of any of the following lesions: flame-shaped haemorrhages, soft exudates or cotton wool spots and papilloedema. Left ventricular hypertrophy (LVH), carotid structural abnormalities, such as plaques and intima media (IM) thickening, and microalbuminuria were diagnosed according to the 2003 ESH/ESC guidelines criteria. RESULTS: Among the whole study population, 33 patients (1.5%) were found to have advanced hypertensive retinopathy. Patients with these retinal lesions were similar to those without for age, body mas index, known duration of hypertension, smoking habit, total serum cholesterol, fasting blood pressure and prevalence of antihypertensive treatment; whereas mean systolic and diastolic blood pressures were higher in the former group. The prevalence rates of LVH, carotid plaques, carotid IM thickening and microalbuminuria in patients with and without retinopathy were 57%, 67%, 69%, 19% and 25%, 47%, 44%, 12%, respectively. In a multivariate logistic regression analysis, advanced retinopathy was significantly associated with LVH (OR = 4.0), carotid IM thickening (OR = 2.9), carotid plaques (OR = 2.8), but not with microalbuminuria. CONCLUSIONS: Our study indicated that: (i) advanced retinopathy is a rare finding in non-diabetic hypertensive patients seen in a specialist setting; (ii) a strong relation exists between retinal microvascular lesions and cardiac and macrovascular markers of TOD.

Prevalence and characteristics of peripheral retinal degeneration in Chinese adults with high myopia: a cross-sectional prevalence survey.

PURPOSE: The purpose of this study was to study the prevalence of peripheral retinal findings in adult Chinese patients with high myopia (refraction < or = -6.00 D) and to investigate the correlation between the retinal lesions and the severity of myopia or axial length. METHODS: A cross-sectional prevalence survey screening was conducted in high ethnic Chinese myopes with refractions equal to or less than -6.00 D. The examinations included cycloplegic refraction, A and B scan ultrasonography, and retinal examinations by indirect ophthalmoscopy with scleral indentation and Goldmann three-mirror contact lens examination. RESULTS: Two hundred thirteen eyes in 213 patients with a mean refraction of -10.10 +/- 4.23 D (range = -6.00 to -27.00 D) were examined. The mean axial length was 26.69 +/- 1.68 mm (range = 25.18-33.62 mm) and the mean age was 33.5 +/- 10.6 years (range = 18-73 years). The most common peripheral retinal finding was pigmentary degeneration (51.2%), followed by lattice degeneration in 12.2% and retinal holes in 7.5% of eyes. A positive correlation was noted between axial length and the lesions of pigmentary degeneration and pavingstone degeneration. The prevalence of retinal holes was 6.4% and 30.0% in eyes with axial length of < 30 mm and > or = 30 mm, respectively (chi-squared test, p = 0.006). CONCLUSION: A high prevalence of peripheral retinal degenerations was found in adult Chinese high myopes. The presence of retinal holes was positively correlated with very high myopia of an axial length of > or = 30 mm.

Taurine deficiency in Newfoundlands fed commercially available complete and balanced diets.

OBJECTIVE: To determine taurine status in a large group of Newfoundlands related by environment, diet, or breeding to a dog with dilated cardiomyopathy and taurine deficiency. DESIGN: Prospective study. ANIMALS: 19 privately owned Newfoundlands between 5 months and 11.5 years old that had been fed commercial dry diets meeting established nutrient recommendations. PROCEDURE: Diet histories were obtained, and blood, plasma, and urine taurine concentrations and plasma methionine and cysteine concentrations were measured. In 8 dogs, taurine concentrations were measured before and after supplementation with methionine for 30 days. Ophthalmic examinations were performed in 16 dogs; echocardiography was performed in 6 dogs that were taurine deficient. RESULTS: Plasma taurine concentrations ranged from 3 to 228 nmol/mL. Twelve dogs had concentrations < 40 nmol/mL and were considered taurine deficient. For dogs with plasma concentrations < 40 nmol/mL, there was a significant linear correlation between plasma and blood taurine concentrations. For dogs with plasma concentrations > 40 nmol/mL, blood taurine concentrations did not vary substantially. Taurine-deficient dogs had been fed lamb meal and rice diets. Retinal degeneration, dilated cardiomyopathy, and cystinuria were not found in any dog examined for these conditions. The taurine deficiency was reversed by a change in diet or methionine supplementation. CONCLUSIONS AND CLINICAL RELEVANCE: Results indicate a high prevalence of taurine deficiency among an environmentally and genetically related cohort of Newfoundlands fed apparently complete and balanced diets. Blood taurine concentrations indicative of taurine deficiency in Newfoundlands may be substantially less than concentrations indicative of a deficiency in cats.

Human T-cell lymphotropic virus type 1-associated retinal vasculitis in children.

PURPOSE: To describe predominant retinal vasculitis in children carrying human T-cell lymphotropic virus type 1 (HTLV-1). METHODS: The authors examined clinical records of patients with HTLV-1-associated uveitis between 1987 and 2001 in Kagoshima University Hospital and reviewed cases of extensive, smoldering retinal vasculitis. RESULTS: Three previously healthy teenagers noted mild visual symptoms and presented with extensive sheathing of retinal vessels, complicated by mild anterior segment inflammation. The retinal vascular disease responded poorly to systemic corticosteroids, had a smoldering course with persistent sheathing of retinal vessels, and eventually resulted in diffuse chorioretinal degeneration. Results of laboratory studies were unremarkable except for the presence of serum antibodies to HTLV-1. One patient developed HTLV-1-associated myelopathy 11 years after the onset of ocular disease. CONCLUSIONS: The retinal vasculitis differed from the retinal vascular changes commonly seen in HTLV-1-associated uveitis. The authors suggest a clinical disease HTLV-1-associated retinal vasculitis that affects young HTLV-1 carriers, characterized by smoldering retinal vasculitis with ultimate retinal degeneration.

[Progressive retinal atrophy in Abyssinian and Somali cats in the Netherlands (1981-2001)]. / Progressieve retina-atrofie bij Abessijnen en Somali's in Nederland (1981-2001).

From 1981 to 2001, 248 Abyssinian and 127 Somali cats in the Netherlands were examined for hereditary eye disease. Distinct ophthalmoscopic signs consistent with hereditary progressive retinal atrophy (PRA) were observed in 11 Abyssinian cats, and subtle signs in 3 Abyssinian cats. A familial relationship was detected in 13 out of 14 of these cats, which supports a hereditary basis to the condition. Distinct funduscopic signs of retinal degeneration were observed at a median age of 4 years. One cat with advanced retinal degeneration was only 7 months old, whereas the remaining 10 cats were between 2 and 12 years old at the time of diagnosis. These differences in the age of onset are suggestive of at least two types of PRA occurring in Abyssinian cats in the Netherlands: a dysplastic, early-onset and a late-onset retinal degeneration. A large-scale and systematic examination programme for hereditary eye disease will be necessary to assess the incidence of PRA in the Dutch population of Abyssinian and Somali cats as a whole, and to provide a basis for a preventive breeding programme.