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Purpose The main objective of this study is to describe the prevalence, degree and risk of corneal involvement, and visual impact in a pediatric population with blepharokeratoconjunctivitis (BKC). Methods Retrospective, observational, case–control study. Clinical records of patients ≤16 years old with BKC seen between 2006 and 2012 were reviewed. The prevalence and relative risk of corneal involvement was evaluated between patients with and without corneal affection through a univariate and multivariate analysis with logistic regression. Visual acuity at presentation and at last follow-up visit was also analyzed. Results One hundred and fourteen children with BKC, with a male-to-female ratio of 1 : 1 and a mean age at diagnosis of 9.13 years. The mean follow-up time was 26.4 (±25) months. Corneal involvement was present in 39.5% of patients, varying from superficial punctate keratitis to perforation. Corneal changes were not seen in children under 4 years old. The risk of corneal affection was greater in patients with photophobia, hordeolum, female gender and asymmetric disease (OR of 2.69, 11.6, 2.35 and 2.77, respectively). The mean best-corrected visual acuity at presentation was 0.20 (corneal affected group), compared to 0.11 (unaffected group; P =0.02). Conclusions Our study showed an older age at time of diagnosis and a worse visual outcome in patients with BKC and corneal disease compared with previous reports. Early diagnosis and detection of risk factors for corneal involvement, as well as adequate treatment, is mandatory to prevent serious long-term visual repercussions in children with BKC.

Aims: To evaluate the efficacy of 50% autologous serum drops against conventional treatment in ocular surface disorders refractory to normal treatments in a prospective randomised crossover trial. Method: Patients fulfilling ophthalmological and haematological entry criteria were randomised to either 3 months of autologous serum 50% followed by 3 months of their conventional treatment, or 3 months of conventional treatment, followed by 3 months of autologous serum. Clinical assessments, including Schirmer’s test, rose Bengal, and fluorescein staining, were carried out on entry and at monthly intervals. Impression cytology was performed at entry, 3 and 6 months. Grading was carried out on degrees of squamous metaplasia and goblet cell density. Subjective comfort was recorded daily using the “faces” scale. These categorical scores were converted to linear measurement using Rasch analysis. Statistical analysis was carried out using Wilcoxon’s signed rank test and ANOVA. Results: 16 patients were recruited with 31 eyes studied. The ocular surface diseases chiefly included Sjögren’s syndrome (n = 6) and keratoconjunctivitis sicca (n = 5). Impression cytology available in 25 of 31 eyes showed significant improvement on serum treatment, p<0.02. Rasch weighted faces scores were statistically significantly better with serum, p<0.01. Conclusion: The results of this randomised study provide further evidence of the beneficial effects of autologous serum in severe ocular surface disorders. For most of these patients, autologous serum was superior to conventional treatment for improving ocular surface health and subjective comfort.

Background One of the most frequent ocular features of systemic sclerosis (SSc) is dry eye disease (DED), which has been identified to occur in 37–79% of patients. Although several studies have found weak or no correlations between symptoms and signs of dry eye, symptoms are often the motivation for seeking eye care, and are therefore a critical outcome measure when assessing treatment effect. The aim of this study is to evaluate the impact of symptoms of dry eye on vision-related quality of life in patients with systemic sclerosis, and to investigate the relation between clinical tests and symptoms of dry eye in these patients. Methods In this cross-sectional study, 45 consecutive patients with SSc were enrolled. For evaluation of the symptoms the “Ocular Surface Disease Index (OSDI)” questionnaire was applied to all patients. After that, all individuals were submitted to a full ophthalmic examination, including the following tests: tear break-up time, Schirmer I, rose bengal staining. Patients were then grouped into dry eye and non-dry eye groups with regard to the diagnosis of dry eye. Mann–Whitney test was used to compare continuous variables, whereas the Fisher exact test was used to compare categorical data between groups. Spearman’s correlation test was used to analyze the correlations between clinical tests and OSDI scores. P values <0.05 were considered significant. Results Dry eye disease was diagnosed in 22 patients (48.9%). Other ocular surface abnormalities found were: blepharitis (40% of the patients), pterygium (15.6%), pinguecula (82.2%), and superficial punctate keratitis (26.7%). Among the 45 patients, 29 patients (64.4%) had symptoms of ocular surface disease. The mean OSDI score was 26.8 ± 25.8 (SD). There were no statistically significant differences in OSDI scores between DED and non-DED patients. No substantive correlations were found between OSDI scores and TBUT, Schirmer I, or rose bengal staining score, and none of the observed correlations reached statistical significance. Conclusion Symptoms of dry eye have a moderate impact on vision-related quality of life in patients with systemic sclerosis and do not correlate well with clinical findings.

To compare the effects of treatment with punctal plugs versus artificial tears on visual function for primary Sjögren’s syndrome with dry eye. Forty-two eyes of 42 patients with primary Sjögren’s syndrome were enrolled and were allocated randomly into artificial tears (AT) group and punctal plugs (PP) group. Ocular Surface Disease Index (OSDI) was used, and fluorescent staining for tear film break-up time (BUT), the Schirmer test I (STI) and contrast sensitivity was performed before treatment and was repeated 3 months after treatment. A follow-up of 3 months was achieved in 40 eyes of 40 patients, including 19 eyes in artificial tears group and 21 eyes in punctal plugs group. Statistically significant improvements were observed in the OSDI scores (AT: 52.6 ± 5.7, 15.9 ± 4.2; PP: 55.8 ± 4.9, 15.1 ± 4.2), corneal fluorescein staining scores (AT: 2.60 ± 1.76, 0.30 ± 0.57; PP: 1.91 ± 1.60, 0.09 ± 0.29), STI (AT: 3.85 ± 2.03, 8.95 ± 2.72; PP: 3.36 ± 1.62, 11.41 ± 2.65), and BUT (AT: 2.60 ± 1.39, 6.00 ± 1.81; PP: 2.27 ± 1.12, 7.82 ± 1.84) after treatment compared to those of pre-treatment. The values of STI (AT: 5.10 ± 1.80; PP: 8.05 ± 1.53) and BUT (AT: 3.40 ± 1.31; PP: 5.68 ± 1.13) in punctal plugs group were significantly more improved than those in the artificial tears group. The medium- and high-level frequencies contrast sensitivities were greatly improved in simulated daylight, night, and glare disability conditions after treatment with artificial tears and punctal plugs. However, the changes in contrast sensitivity did not significantly differ between groups. Both artificial tears and punctal plugs relieved dry eye symptoms, repaired corneal lesions, enhanced tear film stability, and improved contrast sensitivity. Punctal plugs could improve tear film stability and elongate the BUT better than artificial tears.

Background: Toxic epidermal necrolysis (TEN) is a rare, drug-induced disease characterized by epidermal detachment and mucosal involvement. After an acute period, potentially disabling cutaneous and ocular sequels may appear. Although long-term complications are not rare, only few outcome studies are published. Objective: To evaluate the incidence of dermatological and ophthalmological sequels following TEN, to describe its clinical aspects and correlation with acute involvement. Patients and Methods: Eight patients surviving to TEN were submitted to dermatological and ophthalmological observation ranging from 0.5 to 8 years after hospitalization. Cutaneous and ocular involvement, during the acute phase, was retrospectively analysed. Results: Dermatological sequels were observed in 6 patients (75%) corresponding to those with more extensive skin involvement in the acute phase. The most frequent complications were cutaneous dyschromia (62.5%) and nail dystrophies (37.5%). Six patients (75%) had ocular complications with tarsal conjunctiva keratinization in 5 (62.5%) and keratoconjunctivitis sicca in 4 of them (50%). Trichiasis, corneal neovascularization and symblepharon were observed in 1 case. There was no correlation between the severity of acute ocular involvement and long-term complications. Conclusion: Following TEN, most patients have dermatological and ophthalmological sequels that persist for several years.

Background/aims To determine the ophthalmic manifestations of HIV in a cohort of long-term survivors of perinatally acquired HIV. Methods Twenty-two patients with perinatally acquired HIV who were aged ≥12 years were prospectively studied at a university clinic. They underwent complete ophthalmic examinations and fundus photography. Their medical histories, medications and CD4 counts were abstracted from the medical records. To evaluate for keratoconjunctivitis sicca, both HIV patients and 44 healthy controls (matched by age, gender and contact lens wear) underwent Schirmer testing and ocular surface staining. Results Nine male and 13 female HIV patients with mean age of 16.6 years (SD, 3.4) were examined. Of the 22 HIV patients, 21 had been treated with highly active antiretroviral therapy (HAART). Only one patient had a CD4 count nadir of <200 cells/µL. The mean visual acuity of the eyes of the HIV subjects was 20/22 (SD, 1.6 lines). No patient had cytomegalovirus retinitis. Four of the 22 (18%) HIV patients had strabismus. HIV subjects and controls had similar rates of abnormal Schirmer (9% and 14%, p=0.62) and ocular staining scores (p=0.29). Conclusions In the post-HAART era, long-term survivors of perinatally acquired HIV exhibited little vision-threatening disease, but had a high prevalence of strabismus.

Purpose: To study the corneal topographic response to IOP reduction in vernal keratoconjunctivitis (VKC) with steroid-induced glaucoma. Methods: A total of 42 eyes of 21 patients with VKC and steroid-induced glaucoma (Group I) and 66 eyes of 33 patients with VKC without glaucoma (Group II) underwent an evaluation by Orbscan topography. In eyes with glaucoma, the IOP was controlled medically and the corneal topography was repeated at 3 months to evaluate effect on corneal parameters. Results: The mean baseline IOP was 36.40±13.08 mmHg in Group I, 14.67±4.62 mmHg in Group II ( P <0.0001). The IOP after treatment at 3 months follow-up was 15.00±5.41 mmHg in Group I ( P <0.0001). In Group I, the mean maximum Sim K decreased from 44.86±3.21 D to 43.87±2.62 D ( P =0.031) and mean posterior corneal elevation decreased from 64.9±22.36  μ m to 35.7±28.91  μ m at 3 months after reduction of IOP ( P =0.001). There was a significant positive correlation between the reduction in the IOP and the decrease in the posterior corneal elevation ( r =0.664, P =0.001). Conclusion: Eyes with VKC with and without glaucoma have similar corneal topography. Increased IOP associated with steroid-induced glaucoma and VKC may contribute to an increase in the corneal curvature and posterior corneal elevation. These changes may be reversed by a reduction in the IOP with medical therapy.

Background: In severe allergic eye diseases, the breakdown of epithelial barrier function can lead to severe corneal damage such as erosions or ulcers which often resist treatment. Although eosinophils are thought to play a crucial role in corneal tissue damage in severe ocular allergy, the mechanisms of eosinophil recruitment to the cornea has not been fully clarified. Eotaxin has been found in tears of severe allergic patients with corneal ulcer. In this study, we investigated whether the Th2 cytokine interleukin-4 (IL-4) induces eotaxin production in human corneal epithelial cells and keratocytes. Methods: Primary cultures of human corneal epithelial cells and keratocytes were incubated with IL-4 and/or TNF-α for 48 h. Released eotaxin was measured by ELISA, and the eotaxin proteins were visualized by immunocytochemistry. Eotaxin mRNA expression in cultured cells was analyzed by RT-PCR. Results: IL-4 induced eotaxin production in keratocytes in a dose- and time-dependent manner which was enhanced by TNF-α. There was no detectable eotaxin produced by corneal epithelial cells (<5 pg/ml). The cytoplasm of keratocytes incubated with IL-4 stained positively against anti-eotaxin antibodies, while eotaxin mRNA was detected in keratocytes incubated with IL-4. Conclusions: Human corneal keratocytes, but not epithelial cells, are capable of producing eotaxin by stimulation with IL-4. Our results suggest that eotaxin production in keratocytes induced by IL-4 may play an important role in eosinophil recruitment to corneal ulcers in allergic ocular disease. Eotaxin production by keratocytes may explain the severity of allergic disease involving the corneal stroma.

Five outbreaks of infectious keratoconjunctivitis (IKC) affecting alpine chamois and ibex in the western and central Swiss Alps were recorded in 2001 to 2003. Mycoplasma conjunctivae was identified from conjunctival swabs by means of a nested PCR in 27 of the 28 chamois tested. The outbreaks occurred in an area covering 1590 km2. Deep valleys acted as a barrier to the spread of the disease. A total of 409 chamois and 33 ibex with clinical signs of IKC were reported. Most of the chamois were shot, primarily because they were blind or in poor general body condition. Almost a quarter were observed alive, and 16·9 per cent died as a result of IKC. Many of the affected animals were juveniles, and more females than males died of IKC. The disease was more common during the summer and autumn. The chamois affected by IKC were found at altitudes between 550 and 3200 m. The estimated overall mortality was less than 5 per cent, but more than 20 per cent may have died locally. Ibex affected by IKC were recorded in only two outbreaks. In six places, ibex with clinical signs of IKC were found before the first affected chamois appeared in the same area.

O-Acetylated sialic acids have been reported in many sialoglycoproteins where they mediate a variety of immune and other biological events. We have previously demonstrated that the protective mucus barrier on the surface of the canine eye contains sialoglycoproteins. We have also investigated the occurrence of O-acetylated sialic acids in these ocular mucins. Mucus aspirated from the surface of normal dog eyes and those with keratoconjunctivitis sicca (KCS) was fractionated into three pools by density gradient centrifugation. Sialic acids comprised 0.6-0.9% of the dry weight of the mucins isolated. The sialic acid profile in these pools was examined using HPLC. O-Acetylated sialic acids, mainly Neu5,9Ac2, were detected in normal animals and made up 10-30% of the total sialic acids detected. A doubling of the sialic acid content was found in KCS mucins, but the level of 9-O-acetylated sialic acid was reduced below 4% of total. Histological analysis of conjunctival tissue from normal and KCS dogs showed the presence of sialic acids, detected with the alpha(2-6) sialic acid-specific lectin Sambucus nigra, in the goblet cells and corresponding to the staining pattern for MUC5AC, the major ocular-secreted mucin gene product. In KCS animals a disruption of the normal pattern of conjunctival goblet cells was seen with preservation of the pattern of lectin binding observed in normal animals. Thus the data demonstrate the presence of mono-O-Acetylated sialic acids in normal canine ocular mucins and a loss of this population of sialic acids in dry eye disease in spite of a significant increase in total sialic acids in KCS mucin.