title: G6PD deficiency, absence of alpha-thalassemia, and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia.
abstract: Stroke is predicted by abnormally high cerebral velocities by transcranial doppler (TCD). This study aimed at defining predictive factors for abnormally high velocities (>/= 2 m/sec) based on the Créteil pediatric sickle cell anemia (SCA) cohort composed of 373 stroke-free SCA children. alpha genes and beta-globin haplotypes were determined. Biologic parameters were obtained at baseline. alpha-thalassemia was present in 155 of 325 and G6PD deficiency in 36 of 325 evaluated patients. TCD was abnormal in 62 of 373 patients. Multivariate logistic regression analysis showed that G6PD deficiency (odds ratio [OR] = 3.36, 95% confidence interval [CI] 1.10-10.33; P = .034), absence of alpha-thalassemia (OR = 6.45, 95% CI 2.21-18.87; P = .001), hemoglobin (OR per g/dL = 0.63, 95% CI 0.41-0.97; P = .038), and lactate dehydrogenase (LDH) levels (OR per IU/L = 1.001, 95% CI 1.000-1.002; P = .047) were independent risk factors for abnormally high velocities. This study confirms the protective effect of alpha-thalassemia and shows for the first time that G6PD deficiency and hemolysis independently increase the risk of cerebral vasculopathy.
authors.affiliation: Reference Center for Sickle Cell Disease, Department of Pediatrics, Intercommunal Créteil Hospital, Créteil, France. francoise.bernaudin@chicreteil.fr
authors.forename: Françoise
authors.initials: F
authors.lastname: Bernaudin
authors.forename: Suzanne
authors.initials: S
authors.lastname: Verlhac
authors.forename: Sylvie
authors.initials: S
authors.lastname: Chevret
authors.forename: Martine
authors.initials: M
authors.lastname: Torres
authors.forename: Lena
authors.initials: L
authors.lastname: Coic
authors.forename: Cécile
authors.initials: C
authors.lastname: Arnaud
authors.forename: Annie
authors.initials: A
authors.lastname: Kamdem
authors.forename: Isabelle
authors.initials: I
authors.lastname: Hau
authors.forename: Maria
authors.initials: M
authors.lastname: Grazia Neonato
authors.forename: Christophe
authors.initials: C
authors.lastname: Delacourt
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D001783:Blood Flow Velocity / Q000235:genetics; D002560:Cerebrovascular Circulation / Q000235:genetics; D015331:Cohort Studies; D005260:Female; D005914:Globins / Q000032:analysis / Q000235:genetics; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D006461:Hemolysis / Q000235:genetics; D006801:Humans; D006836:Hydro-Lyases / Q000097:blood / Q000235:genetics; D007223:Infant; D008297:Male; D012307:Risk Factors; D020521:Stroke / Q000097:blood / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology; D017585:Ultrasonography, Doppler, Transcranial; D017085:alpha-Thalassemia / Q000097:blood / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*
chemical_list: D005914:Globins; D006836:Hydro-Lyases; C045581:lactate dehydratase