Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia

Glucose‐6‐Phosphate Dehydrogenase Deficiency in Brazilian Children With Sickle Cell Anemia is not Associated With Clinical Ischemic Stroke or High‐Risk Transcranial Doppler

“…Consistent with data from other reports that measured G6PD activity , we did not detect any difference in the mean G6PD activity in groups with and without clinical ischemic stroke or high‐risk TCD. Reports from Créteil (France) have demonstrated the association between G6PD activity and high‐risk TCD and/or vasculopathy on MRA .…”

Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort

“…48 Our findings of an association between G6PD deficiency and abnormal TCD and stenoses seem relevant because increased oxidative stress and decreased nitric oxide levels have also been reported in G6PD-deficient endothelial cells. 49 An association between G6PD deficiency and the risk of abnormal TCD was not found in a United Kingdom cohort 50 ; however, several differences between the studies could explain this discrepancy, as we pointed out in our rebuttal. 50 First, abnormal TCD was defined as TAMMX Ͼ 169 in the United Kingdom cohort and not as Ն 200 cm/s, and second, the follow-up was shorter.…”

“…49 An association between G6PD deficiency and the risk of abnormal TCD was not found in a United Kingdom cohort 50 ; however, several differences between the studies could explain this discrepancy, as we pointed out in our rebuttal. 50 First, abnormal TCD was defined as TAMMX Ͼ 169 in the United Kingdom cohort and not as Ն 200 cm/s, and second, the follow-up was shorter. In addition, we also show that biologic parameters recorded at baseline between 1 and 3 years of age have an important prognostic value, given that independent predictive factors for cerebral vasculopathy risk were high reticulocyte counts and high LDH levels, demonstrating the impact of hemolysis as a risk factor for cerebral vasculopathy.…”

Advances in understanding the pathogenesis of cerebrovascular vasculopathy in sickle cell anaemia

“…Despite a trend towards more severe anaemia in patients with G6PD deficiency (baseline haemoglobin: 76 ± 13 vs. 81 ± 12 g/l, P = 0·13), no significant difference was found in the haemolytic rate (reticulocyte count and LDH) of SCA patients with or without G6PD deficiency (Bernaudin et al , ), suggesting that G6PD deficiency increases the risk for cerebral vasculopathy by a mechanism other than haemolysis or anaemia. Such association between G6PD deficiency and abnormal TCD was not found in another cohort study (Rees et al , ); however, several differences between the studies could explain this discrepancy, as pointed out in the rebuttal. In particular the cut‐off defining abnormal velocities was ≥ 200 cm/s in the earlier cohort (Bernaudin et al , ) vs. 170 cm/s in the cohort reported by Rees et al () cohort.…”

“…Such association between G6PD deficiency and abnormal TCD was not found in another cohort study (Rees et al , ); however, several differences between the studies could explain this discrepancy, as pointed out in the rebuttal. In particular the cut‐off defining abnormal velocities was ≥ 200 cm/s in the earlier cohort (Bernaudin et al , ) vs. 170 cm/s in the cohort reported by Rees et al () cohort. A new analysis concerning the Cooperative Study of Sickle Cell Disease (CSSCD) cohort (800 males with G6PD isoenzymes and activity performed) was recently reported (Miller et al , ), which found no higher stroke risk among the patients with G6PD deficiency.…”