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.
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.
Bernaudin
Françoise
F
Reference Center for Sickle Cell Disease, Department of Pediatrics, Intercommunal Créteil Hospital, Créteil, France. francoise.bernaudin@chicreteil.fr
Verlhac
Suzanne
S
Chevret
Sylvie
S
Torres
Martine
M
Coic
Lena
L
Arnaud
Cécile
C
Kamdem
Annie
A
Hau
Isabelle
I
Grazia Neonato
Maria
M
Delacourt
Christophe
C
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*
D005914:Globins; D006836:Hydro-Lyases; C045581:lactate dehydratase