Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

Abstract Background Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and ev...

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Main Authors: Mohammed Nawaiseh, Allaa Roto, Yara Nawaiseh, Mohammad Salameh, Rund Haddadin, Lana Mango, Hussam Nawaiseh, Doha Alsaraireh, Qais Nawaiseh, Saif Aldeen AlRyalat, Amer Alwreikat, David J. Ramsey, Nakhleh Abu-Yaghi
Format: Article
Language:English
Published: BMC 2022-09-01
Series:International Journal of Retina and Vitreous
Subjects:
Online Access:https://doi.org/10.1186/s40942-022-00419-8
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author Mohammed Nawaiseh
Allaa Roto
Yara Nawaiseh
Mohammad Salameh
Rund Haddadin
Lana Mango
Hussam Nawaiseh
Doha Alsaraireh
Qais Nawaiseh
Saif Aldeen AlRyalat
Amer Alwreikat
David J. Ramsey
Nakhleh Abu-Yaghi
author_facet Mohammed Nawaiseh
Allaa Roto
Yara Nawaiseh
Mohammad Salameh
Rund Haddadin
Lana Mango
Hussam Nawaiseh
Doha Alsaraireh
Qais Nawaiseh
Saif Aldeen AlRyalat
Amer Alwreikat
David J. Ramsey
Nakhleh Abu-Yaghi
author_sort Mohammed Nawaiseh
collection DOAJ
description Abstract Background Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice. Methods This research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system. Results Of the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021). Conclusion In this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.
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spelling doaj.art-dc33cbef9744431faf33e6df06cb5b532022-12-22T04:25:56ZengBMCInternational Journal of Retina and Vitreous2056-99202022-09-018111010.1186/s40942-022-00419-8Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell DiseaseMohammed Nawaiseh0Allaa Roto1Yara Nawaiseh2Mohammad Salameh3Rund Haddadin4Lana Mango5Hussam Nawaiseh6Doha Alsaraireh7Qais Nawaiseh8Saif Aldeen AlRyalat9Amer Alwreikat10David J. Ramsey11Nakhleh Abu-Yaghi12King Hussein Cancer CenterAl Bahar Eye center, Ibn Sina Hospital, Ministry of HealthFaculty of Medicine, Jordan University of Science and TechnologyHamad Medical CorporationSchool of Medicine, The University of JordanSchool of Medicine, The University of JordanSchool of Medicine, The University of JordanSchool of Medicine, The University of JordanSchool of Medicine, The University of JordanDepartment of Special Surgery, Ophthalmology Division, School of Medicine, The University of JordanDivision of Ophthalmology, Department of Surgery, Lahey Hospital & Medical CenterDivision of Ophthalmology, Department of Surgery, Lahey Hospital & Medical CenterDepartment of Special Surgery, Ophthalmology Division, School of Medicine, The University of JordanAbstract Background Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice. Methods This research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system. Results Of the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021). Conclusion In this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.https://doi.org/10.1186/s40942-022-00419-8Sickle cell diseaseSickle cell retinopathyProliferative sickle cell retinopathyHemoglobin FSmoking
spellingShingle Mohammed Nawaiseh
Allaa Roto
Yara Nawaiseh
Mohammad Salameh
Rund Haddadin
Lana Mango
Hussam Nawaiseh
Doha Alsaraireh
Qais Nawaiseh
Saif Aldeen AlRyalat
Amer Alwreikat
David J. Ramsey
Nakhleh Abu-Yaghi
Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
International Journal of Retina and Vitreous
Sickle cell disease
Sickle cell retinopathy
Proliferative sickle cell retinopathy
Hemoglobin F
Smoking
title Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
title_full Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
title_fullStr Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
title_full_unstemmed Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
title_short Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease
title_sort risk factors associated with sickle cell retinopathy findings from the cooperative study of sickle cell disease
topic Sickle cell disease
Sickle cell retinopathy
Proliferative sickle cell retinopathy
Hemoglobin F
Smoking
url https://doi.org/10.1186/s40942-022-00419-8
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