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

摘要

Cerebrovascular disease is an important complication in children with sickle.cell anemia (SCA). Hemolysis is increasingly implicated in the pathogenesis. Recently, Bernaudin et al found that glucose 6 phosphate dehydrogenase (G6PD) deficiency was associated with high transcranial Doppler (TCD) velocities. We have retrospectively analyzed data from children with SCA who have been seen at our hospital over the last 6 years, to assess the proposed link between G6PD deficiency and cerebrovascular disease. TCD imaging was performed using standard protocols. A 2-stage assay was used to quantitate G6PD activity, and deficiency was defined as activity less than 5.2 IU/g hemoglobin. No attempt was made to identify female heterozygotes. Data were collected as part of an audit. Statistical analysis was performed using SPSS Statistics 17.0 (SPSS). Of 329 children with SCA, 218 had G6PD results available. Twenty-four (11.0%) had G6PD deficiency. Five of 97 females and 19 of 121 males (x~2 = 6.1, P = .013) had the deficiency, the excess of males reflecting X-linked inheritance. Twenty-seven children with known G6PD status had cerebrovascular disease (10 with averaged mean of the maximum velocity [TAMMX] > 200 cm/ sec, 13 with TAMMX > 169 cm/sec but < 200 cm/sec, and 4 with strokes and stenosed vessels on magnetic resonance (MR) angiog-raphy but inadequate TCDs). TCD imaging tends to give lower readings than TCD, although the exact equivalence is not defined, and we have classified a TAMMX greater than 169 cm/sec as abnormal, requiring further investigation or action. There was no significant difference between the mean TAMMX in females versus males (130 vs 126 cm/sec, P = .297, t test).