March 2012
Volume 53, Issue 14
Free
ARVO Annual Meeting Abstract  |   March 2012
Severity of Proliferative Sickle Cell Retinopathy Associated With the Clinical and Laboratory Factors inPatients With Sickle Cell Homozygous Sickle Cell (SS) and Hemoglobin C (SC) Disease
Author Affiliations & Notes
  • Franck Lalloum
    Department of Ophthalmology- Hôpital Intercommunal de Créteil, Université Henri Mondor - Paris XII, CRETEIL, France
  • Nicolas Leveziel
    Department of Ophthalmology- Hôpital Intercommunal de Créteil, Université Henri Mondor - Paris XII, CRETEIL, France
  • Sylvie Bastuji-Garin
    Hôpital Henri-Mondor, Department of Clinical Research and Public Health, CRETEIL, France
  • Giuseppe Querques
    Department of Ophthalmology- Hôpital Intercommunal de Créteil, Université Henri Mondor - Paris XII, CRETEIL, France
  • Pascale Benlian
    UPMC Univ Paris 6, Biochemistry and Molecular Biology Department, CRETEIL, France
  • Michel Binaghi
    Department of Ophthalmology- Hôpital Intercommunal de Créteil, Université Henri Mondor - Paris XII, CRETEIL, France
  • Frederic Galacteros
    Hôpital Henri Mondor, National Referral Center for Adult Sickle-Cell Disease, Red Cell Genetic Disease Unit, CRETEIL, France
  • Eric Souied
    Department of Ophthalmology- Hôpital Intercommunal de Créteil, Université Henri Mondor - Paris XII, CRETEIL, France
  • Footnotes
    Commercial Relationships  Franck Lalloum, None; Nicolas Leveziel, None; Sylvie Bastuji-Garin, None; Giuseppe Querques, None; Pascale Benlian, None; Michel Binaghi, None; Frederic Galacteros, None; Eric Souied, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science March 2012, Vol.53, 990. doi:
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      Franck Lalloum, Nicolas Leveziel, Sylvie Bastuji-Garin, Giuseppe Querques, Pascale Benlian, Michel Binaghi, Frederic Galacteros, Eric Souied; Severity of Proliferative Sickle Cell Retinopathy Associated With the Clinical and Laboratory Factors inPatients With Sickle Cell Homozygous Sickle Cell (SS) and Hemoglobin C (SC) Disease. Invest. Ophthalmol. Vis. Sci. 2012;53(14):990.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : Sickle cell disease (SCD) is among the most common, life-threatening monogenic disorders in the world. Proliferative sickle cell retinopathy (PSCR) is the most frequent vision-threatening complication of sickle cell disease (SCD). We studied the relationship between the severity of sickle cell retinopathy in heterozygous (SC) or homozygous (SS) adult SCD patients and the clinical and laboratory data obtained during visits to a national SCD referral center.

Methods: : Retrospective longitudinal analysis included 942 SCD patients (313 patients with SC and 629 with SS disease) with ophthalmologic evaluations who were followed over a 19-year period by a multidisciplinary team in a referral center. Goldberg classification was used to gratuate PSCR. We identified patient and SCD characteristics associated with sickle cell retinopathy severity using multinomiallogistic-regression models.

Results: : Multivariate analysis associated severe PSCR forms (stages III-V) with older age (p = 0.032), pulmonary involvement (documented pulmonary hypertension with pulmonary arterial pressure ≥ 40 mm Hg, restrictive syndrome > 20%, or previous history of pulmonary embolism diagnosed by vascular imaging) (p = 0.029), deafness or tinnitus (p = 0.026), and no history of osteomyelitis (p = 0.013) for SC patients; and with older age (p < 0.001), male sex (p = 0.003), andacute pyelonephritis (p = 0.04) for SS patients.

Conclusions: : The model of severe PSCR versus no PSCR showed good calibration and discrimination for SC and SS patients. Analysis of this large SCD population enabled us to establish several significant associations between retinal features of PSCR and other clinical features of SCD. Awareness of the clinical and laboratory factors significantly associated with severe PSCR in patients with SC or SS SCD may contribute to improved preventive strategies. Prospective studies are needed to establish a clinical scoring system that can predict with acceptable sensitivityand specificity the risk of developing severe PSCR.

Keywords: retina • neovascularization • clinical (human) or epidemiologic studies: prevalence/incidence 
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