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      Retinal findings in membranoproliferative glomerulonephritis

      case-report

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          Abstract

          Purpose

          To assess the evolution of retinal findings in patients with membranoproliferative glomerulonephritis (MPGN) by funduscopy, intravenous fluorescein angiography and optical coherence tomography.

          Observations

          Three women and one man were followed for a period of 1.5–37 years. Four patients (8 eyes) had drusen detected at first fundus exam at age 24, 29, 50 and 55. Three patients (6 eyes) had diffuse thickening of Bruch's membrane, and two patients (3 eyes) had detachment of the retinal pigment epithelium with serous retinal detachment. Drusen tended to widen over a period of 10-year follow-up in one case.

          Conclusions and importance

          Drusen remain the ocular stigmata for MPGN occuring at an early age. The retinal disease is progressive with gradual thickening of Bruch's membrane and occurrence of retinal pigment epithelium detachment.

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          Most cited references30

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          Drusen associated with aging and age-related macular degeneration contain proteins common to extracellular deposits associated with atherosclerosis, elastosis, amyloidosis, and dense deposit disease.

          Age-related macular degeneration (AMD), a blinding disorder that compromises central vision, is characterized by the accumulation of extracellular deposits, termed drusen, between the retinal pigmented epithelium and the choroid. Recent studies in this laboratory revealed that vitronectin is a major component of drusen. Because vitronectin is also a constituent of abnormal deposits associated with a variety of diseases, drusen from human donor eyes were examined for compositional similarities with other extracellular disease deposits. Thirty-four antibodies to 29 different proteins or protein complexes were tested for immunoreactivity with hard and soft drusen phenotypes. These analyses provide a partial profile of the molecular composition of drusen. Serum amyloid P component, apolipoprotein E, immunoglobulin light chains, Factor X, and complement proteins (C5 and C5b-9 complex) were identified in all drusen phenotypes. Transcripts encoding some of these molecules were also found to be synthesized by the retina, retinal pigmented epithelium, and/or choroid. The compositional similarity between drusen and other disease deposits may be significant in view of the recently established correlation between AMD and atherosclerosis. This study suggests that similar pathways may be involved in the etiologies of AMD and other age-related diseases.
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            C3 glomerulopathy: a new classification.

            Several distinct pathological patterns of glomerular inflammation are associated with abnormal regulation of the complement system, specifically, with dysregulation of the alternative pathway of the complement system. However, these conditions share the pathological finding of complement C3 (C3) deposited within the glomerulus in the absence of substantial immunoglobulin. This finding has alerted us and others to the possible presence of genetic and acquired complement dysregulation in individual patients. This article summarizes our current understanding of the relationship between dysregulation of the complement system and glomerular inflammation. Here, we suggest that glomerular pathologies that are characterized by the isolated deposition of C3 could usefully be classified by the term C3 glomerulopathy. In our view, this classification would alert the pathologist and nephrologist to the importance of screening for acquired and genetic abnormalities in complement regulation. In the future, it could help to identify individuals who might benefit from therapeutic inhibition of the complement system.
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              Eculizumab in a patient with dense-deposit disease.

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                Author and article information

                Contributors
                Journal
                Am J Ophthalmol Case Rep
                Am J Ophthalmol Case Rep
                American Journal of Ophthalmology Case Reports
                Elsevier
                2451-9936
                22 June 2017
                September 2017
                22 June 2017
                : 7
                : 83-90
                Affiliations
                [a ]Department of Ophthalmology, American University of Beirut, Beirut, Lebanon
                [b ]Department of Ophthalmology, Rafic Hariri University Hospital, Beirut, Lebanon
                [c ]Department of Ophthalmology and Visual Sciences, Federal University of Sao Paulo, Sao Paulo, Brazil
                [d ]Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, USA
                [e ]Instituto de Olhos e Laser de Belém, Federal University of Sao Paulo, Sao Paulo, Brazil
                [f ]Department of Ophthalmology, Complejo Hospitalario Universitario de Canarias, Tenerife, Canary Islands, Spain
                [g ]Department of Ophthalmology, McGill University, Montreal, Canada
                Author notes
                []Corresponding author. American University of Beirut, Beirut POB 1136044, Lebanon.American University of BeirutBeirutPOB 1136044Lebanon ammansourmd@ 123456gmail.com
                Article
                S2451-9936(16)30282-1
                10.1016/j.ajoc.2017.06.011
                5722170
                143c29b0-2e29-4fc5-9e1a-b637ff9c08f0
                © 2017 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 20 November 2016
                : 10 April 2017
                : 20 June 2017
                Categories
                Case report

                drusen,macular degeneration,membranoproliferative glomerulonephritis,retinal pigment epithelial detachment

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