Sickle Retinopathy in a Person with Hemoglobin S/New York Disease

A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not pr...

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Main Authors: Donovan Calder, Maryse Etienne-Julan, Marc Romana, Naomi Watkins, Jennifer M. Knight-Madden
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Genetics
Online Access:http://dx.doi.org/10.1155/2012/136582
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author Donovan Calder
Maryse Etienne-Julan
Marc Romana
Naomi Watkins
Jennifer M. Knight-Madden
author_facet Donovan Calder
Maryse Etienne-Julan
Marc Romana
Naomi Watkins
Jennifer M. Knight-Madden
author_sort Donovan Calder
collection DOAJ
description A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ophthalmological findings, the laboratory diagnosis, and possible pathophysiology of this disorder are discussed. Persons diagnosed with sickle cell trait who present with symptoms of sickle cell disease may benefit from specific screening for this variant.
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series Case Reports in Genetics
spelling doaj-art-ba0effb2f5ad4d1693c510b443ad86302025-08-20T02:19:26ZengWileyCase Reports in Genetics2090-65442090-65522012-01-01201210.1155/2012/136582136582Sickle Retinopathy in a Person with Hemoglobin S/New York DiseaseDonovan Calder0Maryse Etienne-Julan1Marc Romana2Naomi Watkins3Jennifer M. Knight-Madden4Department of Surgery, Radiology, Anaesthetics, and Intensive Care, Faculty of Medical Sciences, University of the West Indies, Mona, Kingston 7, JamaicaUniversite des Antilles et de la Guyane, Centre Hospitalier Universitaire (CHU), Pointe-a-Pitre, UMR S 458 Inserm, 97159 Guadeloupe, FranceInserm U763, Pointe-à-Pitre, 97159 Guadeloupe, FranceSickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston 7, JamaicaSickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston 7, JamaicaA patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ophthalmological findings, the laboratory diagnosis, and possible pathophysiology of this disorder are discussed. Persons diagnosed with sickle cell trait who present with symptoms of sickle cell disease may benefit from specific screening for this variant.http://dx.doi.org/10.1155/2012/136582
spellingShingle Donovan Calder
Maryse Etienne-Julan
Marc Romana
Naomi Watkins
Jennifer M. Knight-Madden
Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
Case Reports in Genetics
title Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
title_full Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
title_fullStr Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
title_full_unstemmed Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
title_short Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
title_sort sickle retinopathy in a person with hemoglobin s new york disease
url http://dx.doi.org/10.1155/2012/136582
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