From the Muhimbili Wellcome Programme, Dar es Salaam, Tanzania (E.N.K., E.M., D.S., J.K., D.A.N., S.E.C., C.R.J.C.N.).
Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania (E.N.K., M.M., D.S., C.R.J.C.N.).
Stroke. 2019 Jul;50(7):1719-1726. doi: 10.1161/STROKEAHA.118.018920. Epub 2019 Jun 14.
Background and Purpose- We determined prevalences of neurological complications, vascular abnormality, and infarction in Tanzanian children with sickle cell disease. Methods- Children with sickle cell disease were consecutively enrolled for transcranial Doppler; those with slightly elevated (>150 cm/s), low (<50 cm/s) or absent cerebral blood flow velocity (CBFv) were invited for brain magnetic resonance imaging and magnetic resonance angiography. Results- Of 200 children (median age 9; range 6-13 years; 105 [2.5%] boys), 21 (11%) and 15 (8%) had previous seizures and unilateral weakness, respectively. Twenty-eight (14%) had elevated and 39 (20%) had low/absent CBFv, all associated with lower hemoglobin level, but not higher indirect bilirubin level. On multivariable analysis, CBFv>150 cm/s was associated with frequent painful crises and low hemoglobin level. Absent/low CBFv was associated with low hemoglobin level and history of unilateral weakness. In 49 out of 67 children with low/absent/elevated transcranial Doppler undergoing magnetic resonance imaging, 43% had infarction, whereas 24 out of 48 (50%) magnetic resonance angiographies were abnormal. One had hemorrhagic infarction; none had microbleeds. Posterior circulation infarcts occurred in 14%. Of 11 children with previous seizure undergoing magnetic resonance imaging, 10 (91%) had infarction (5 silent) compared with 11 out of 38 (29%) of the remainder ( P=0.003). Of 7 children with clinical stroke, 2 had recurrent stroke and 3 died; 4 out of 5 had absent CBFv. Of 193 without stroke, 1 died and 1 had a stroke; both had absent CBFv. Conclusions- In one-third of Tanzanian children with sickle cell disease, CBFv is outside the normal range, associated with frequent painful crises and low hemoglobin level, but not hemolysis. Half have abnormal magnetic resonance angiography. African children with sickle cell disease should be evaluated with transcranial Doppler; those with low/absent/elevated CBFv should undergo magnetic resonance imaging/magnetic resonance angiography.
背景与目的-我们确定了坦桑尼亚镰状细胞病患儿的神经并发症、血管异常和梗死发生率。方法-连续招募镰状细胞病患儿进行经颅多普勒检查；那些脑血流速度（CBFv）稍高（>150cm/s）、低（<50cm/s）或缺失的患儿被邀请进行脑磁共振成像和磁共振血管造影。结果-在 200 名儿童（中位年龄 9 岁；范围 6-13 岁；105[2.5%]名男孩）中，分别有 21 名（11%）和 15 名（8%）患儿有既往癫痫发作和单侧无力。28 名（14%）患儿 CBFv 升高，39 名（20%）患儿 CBFv 降低/缺失，均与较低的血红蛋白水平相关，但与较高的间接胆红素水平无关。多变量分析显示，CBFv>150cm/s 与频繁的疼痛危象和低血红蛋白水平相关。CBFv 降低/缺失与低血红蛋白水平和单侧无力史相关。在 67 名低/缺失/升高经颅多普勒检查的患儿中，有 49 名进行了磁共振成像，其中 43%有梗死，而 48 名磁共振血管造影中有 24 名（50%）异常。1 例为出血性梗死；无一例有微出血。后循环梗死占 14%。在 11 名有既往癫痫发作而进行磁共振成像的患儿中，10 名（91%）有梗死（5 例为无症状性梗死），而在其余 38 名患儿中，有 11 名（29%）（P=0.003）。在 7 名有临床脑卒中的患儿中，2 名有复发性脑卒中，3 名死亡；4 名患儿 CBFv 缺失。在 193 名无脑卒中的患儿中，1 名死亡，1 名发生脑卒中；2 名患儿 CBFv 缺失。结论-在三分之一的坦桑尼亚镰状细胞病患儿中，CBFv 处于正常范围之外，与频繁的疼痛危象和低血红蛋白水平相关，但与溶血无关。半数患儿磁共振血管造影异常。非洲镰状细胞病患儿应进行经颅多普勒检查；那些 CBFv 降低/缺失/升高的患儿应进行磁共振成像/磁共振血管造影检查。
