Clinical and Laboratory Factors Associated With the Severity of Proliferative Sickle Cell Retinopathy in Patients With Sickle Cell Hemoglobin C (SC) and Homozygous Sickle Cell (SS) Disease
| Autor: | Gabriel Coscas, Pascale Benlian, Eric H Souied, Giuseppe Querques, Gisèle Soubrane, Nicolas Leveziel, Franck Lalloum, Frédéric Galactéros, M. Binaghi, Dora Bachir, Sylvie Bastuji-Garin |
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| Přispěvatelé: | Leveziel, N, Bastuji Garin, S, Lalloum, F, Querques, Giuseppe, Benlian, P, Binaghi, M, Coscas, G, Soubrane, G, Bachir, D, Galacteros, F, Souied, Eh |
| Rok vydání: | 2011 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Adolescent Sickle Cell-SS Disease Sickle cell hemoglobin C Cell Disease Gastroenterology Young Adult Retinal Diseases hemic and lymphatic diseases Internal medicine medicine Humans In patient Longitudinal Studies Sickle cell retinopathy Child Aged Retrospective Studies business.industry General Medicine Middle Aged Logistic Models medicine.anatomical_structure Multivariate Analysis Female Hemoglobin SC Disease Complication business |
| Zdroj: | Europe PubMed Central |
| Popis: | Proliferative sickle cell retinopathy (PSCR) is the most frequent vision-threatening complication of sickle cell disease (SCD). We investigated 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. This 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. PSCR was graded using the Goldberg classification. We identified patient and SCD characteristics associated with sickle cell retinopathy severity using multinomial logistic-regression models. 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), and acute pyelonephritis (p = 0.04) for SS patients. The model of severe PSCR versus no PSCR showed good calibration and discrimination for SC and SS patients. 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. (Medicine 2011;90: 372-378) |
| Databáze: | OpenAIRE |
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