G6PD deficiency, absence of α-thalassemia, and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia

Author:

Bernaudin Françoise1,Verlhac Suzanne2,Chevret Sylvie3,Torres Martine4,Coic Lena1,Arnaud Cécile1,Kamdem Annie1,Hau Isabelle1,Grazia Neonato Maria5,Delacourt Christophe1

Affiliation:

1. Reference Center for Sickle Cell Disease, Department of Pediatrics, and

2. Medical Imagerie, Department of Radiology, Intercommunal Créteil Hospital, Créteil, France;

3. Department of Statistics, St-Louis Hospital, Paris, France;

4. Pediatrics, University of Southern California, Los Angeles; and

5. Hematology, Molecular Biology, Tenon Hospital, Paris, France

Abstract

AbstractStroke 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. α genes and β-globin haplotypes were determined. Biologic parameters were obtained at baseline. α-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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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