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      Clinical spectrum of pseudoexfoliation syndrome—An electronic records audit

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          Abstract

          Purpose

          To evaluate different clinical variants of pseudoexfoliation syndrome and their risk of developing ocular hypertension (OHT) or glaucoma (PXG)

          Design

          Cross sectional hospital based study.

          Setting

          All patients seen at glaucoma services of a tertiary eye care center in east India.

          Methods

          Electronic medical records search of hospital database including consecutive new and old cases seen during April 2013 to March 2015 was done to retrieve case sensitive words including pseudoexfoliation, PXF, PEX, PXG and pseudoexfoliative glaucoma over any part of the clinical electronic sheet of the patient. All demographic and clinical details including laterality, the pattern of deposits, need for medicines and disc damage at presentation was compared in eyes with radial pigmentary, classical or combined forms of PXF phenotypes.

          Results

          Of 110313 PXF patients seen during the period of 2013–2015, a total of 2297 eyes of 1150 PXF patients were identified including 525 unilateral PXF (meaning a total of 1775 PXF eyes with 625 patients having bilateral disease, n = 1250 eyes, other clinically normal eye, n = 522) at presentation. Of 525 unilateral PXF eyes, 105 had OHT and 131 had glaucoma while bilateral cases had more >50% (675 eyes of 1250 eyes) with glaucoma. Glaucoma with significant changes in IOP with or without disc damage was seen in 32% of pigmentary and 39% of classical PXF forms with eyes with combined forms of PXF having around 50% with glaucoma at presentation compared to other forms, p<0.001.

          Conclusion

          Different phenotypic variants of PXF in this Indian cohort was associated with 30–50% risk of OHT or glaucoma respectively. Adequate care is required while examining the pattern of PXF in each case to prognosticate each patient/eye.

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          Most cited references27

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          Pseudoexfoliation syndrome for the comprehensive ophthalmologist. Intraocular and systemic manifestations.

          Renewed interest in pseudoexfoliation syndrome (PEX) may be attributed to an increased awareness of many clinical risks not only for open-angle glaucoma and its recent recognition as a generalized disorder. This review summarizes the range of intraocular and extraocular manifestations. Involvement of all tissues of the anterior segment of the eye results in a spectrum of intraocular complications that have management implication for all practicing ophthalmologists. The study design was a review. Clinical diagnosis depends on biomicroscopy, biocytology, and laser-tyndallometry. Laboratory research methods range from light and electron microscopy, to immunohistochemical and molecular biologic approaches. Clinical-histopathologic correlations focus on the involvement of lens (PEX-phacopathy), zonular apparatus (zonulopathy), ciliary body (cyclopathy), iris (iridopathy), trabecular meshwork (trabeculopathy), and cornea (corneal endotheliopathy) leading to the following complications: (1) open-angle glaucoma as well as angle-closure glaucoma due to pupillary and ciliary block; (2) phacodonesis, lens dislocation, and increased incidence of vitreous loss in extracapsular cataract surgery caused by alterations of the zonular apparatus and its insertion into the ciliary body and lens; (3) blood-aqueous barrier breakdown (pseudouveitis), anterior chamber hypoxia, iris stromal hemorrhage, pigment epithelial melanin dispersion, poor or asymmetric pupillary dilatation, and formation of posterior synechiae due to involvement of all cell populations of the iris; and (4) early diffuse corneal endothelial decompensation explained by a damaged and numerically reduced endothelium. In view of the multitude of clinical complications, PEX is of relevance to comprehensive ophthalmologists, including specialists in glaucoma, cataract, cornea, neuro-ophthalmology, and retina. Special attention to the risks associated with PEX is advised before, during, and after surgery.
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            The relationship between glaucoma and pseudoexfoliation: the Blue Mountains Eye Study.

            To quantify the relationship between pseudoexfoliation (PXF) and open-angle glaucoma, ocular hypertension, and intraocular pressure (IOP) in a defined older population. A cross-sectional study of 3654 people aged 49 to 97 years identified subjects with PXF during slitlamp examination. The IOP was measured by applanation tonometry. Glaucoma was diagnosed from characteristic visual field loss combined with optic disc cupping and rim thinning, without reference to IOP. Ocular hypertension was diagnosed if IOP was greater than 21 mm Hg in either eye, without field and disc changes. General estimating equation models were used to assess associations between eyes with PXF and glaucoma or ocular hypertension. Pseudoexfoliation was diagnosed in 2.3% of subjects, and both prevalence and bilaterality increased with age. Glaucomatous damage was present in 14.2% of eyes with PXF compared with 1.7% of eyes without PXF (age- and sex-adjusted odds ratio (OR), 5.0; 95% confidence interval (CI), 2.6-9.6). This was almost unchanged (OR, 4.8) after adjustment for glaucoma risk factors and was also relatively unaffected by IOP adjustment (OR, 3.7; 95% CI, 1.8-7.6). For subjects with PXF, the relationship with glaucoma persisted, but was weaker (OR, 2.3; 95% CI, 1.0-5.0) in the multivariate model. However, the population attributable risk from PXF was only 2.7%. Ocular hypertension was also more frequent in eyes with PXF (9.3%) than in eyes without PXF (3.1%) but was of borderline significance in the multivariate model (OR, 2.3; 95% CI, 0.9-5.7). This study confirmed the strong relationship between glaucoma and PXF. Subjects with PXF had an increased risk of glaucoma, while eyes with PXF had a higher risk, which was independent of other glaucoma risk factors, including IOP.
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              Major review: Exfoliation syndrome; advances in disease genetics, molecular biology, and epidemiology

              Exfoliation syndrome (XFS) is a common age-related disorder that leads to deposition of extracellular fibrillar material throughout the body. The most recognized disease manifestation is exfoliation glaucoma (XFG), which is a common cause of blindness worldwide. Recent developments in XFS genetics, cell biology and epidemiology have greatly improved our understanding of the etiology of this complex inherited disease. This review summarizes current knowledge of XFS pathogenesis, identifies gaps in knowledge, and discusses areas for future research.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: Project administrationRole: ResourcesRole: SupervisionRole: ValidationRole: VisualizationRole: Writing – original draftRole: Writing – review & editing
                Role: ConceptualizationRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: Project administrationRole: SupervisionRole: ValidationRole: VisualizationRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administrationRole: SupervisionRole: ValidationRole: Writing – review & editing
                Role: Data curationRole: InvestigationRole: MethodologyRole: Supervision
                Role: Data curationRole: InvestigationRole: MethodologyRole: ResourcesRole: Writing – review & editing
                Role: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administrationRole: ResourcesRole: SupervisionRole: ValidationRole: VisualizationRole: Writing – review & editing
                Role: Data curationRole: InvestigationRole: MethodologyRole: ResourcesRole: SupervisionRole: VisualizationRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                27 October 2017
                2017
                : 12
                : 10
                : e0185373
                Affiliations
                [001]Glaucoma Service, LV Prasad Eye Institute, Patia, Bhubaneswar, Odisha, India
                Universita degli Studi di Firenze, ITALY
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                http://orcid.org/0000-0001-8120-8740
                Article
                PONE-D-17-13123
                10.1371/journal.pone.0185373
                5659605
                29077713
                89780fdd-792b-49aa-a896-54138659bc6a
                © 2017 Rao et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 4 April 2017
                : 12 September 2017
                Page count
                Figures: 2, Tables: 4, Pages: 11
                Funding
                Funded by: DBT wellcome trust
                Award ID: IA/CPHI/15/1/502031
                Award Recipient :
                This work was funded in part by the DBT Wellcome Trust grant, ref no IA/CPHI/15/1/502031. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Biology and Life Sciences
                Anatomy
                Head
                Eyes
                Medicine and Health Sciences
                Anatomy
                Head
                Eyes
                Biology and Life Sciences
                Anatomy
                Ocular System
                Eyes
                Medicine and Health Sciences
                Anatomy
                Ocular System
                Eyes
                Medicine and Health Sciences
                Ophthalmology
                Eye Diseases
                Glaucoma
                Medicine and Health Sciences
                Ophthalmology
                Eye Diseases
                Biology and Life Sciences
                Anatomy
                Ocular System
                Ocular Anatomy
                Lens (Anatomy)
                Medicine and Health Sciences
                Anatomy
                Ocular System
                Ocular Anatomy
                Lens (Anatomy)
                Medicine and Health Sciences
                Ophthalmology
                Medicine and Health Sciences
                Vascular Medicine
                Blood Pressure
                Hypertension
                Biology and Life Sciences
                Neuroscience
                Sensory Perception
                Vision
                Biology and Life Sciences
                Psychology
                Sensory Perception
                Vision
                Social Sciences
                Psychology
                Sensory Perception
                Vision
                Physical Sciences
                Materials Science
                Materials by Attribute
                Pigments
                Custom metadata
                All relevant data are within the paper and its Supporting Information files.

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