Department of Pediatrics, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria.
Department of Pediatrics, Center of Excellence in Sickle Cell Disease, Vanderbilt University School of Medicine, Nashville, Tennessee.
Pediatr Neurol. 2019 Jun;95:73-78. doi: 10.1016/j.pediatrneurol.2019.01.008. Epub 2019 Jan 17.
To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital.
The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke.
Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Follow-up was a median of 1.04 years (interquartile range 0.43 to 1.83 years) to either July 2017 or a second stroke, corresponding to an initial stroke incidence rate of 0.88 per 100 patient-years. Eight children had a recurrent stroke, six of whom were prescribed hydroxyurea 20 mg/kg/day by two months after initial stroke. Two children died. Six of the recurrent strokes occurred within six months of the initial stroke, two before hydroxyurea prescription. The stroke recurrence rate was 17.4 events per 100 patient-years. Adherence was approximately 60%, partly because families had to pay for hydroxyurea. Stroke incidence is probably underestimated because despite formal training for stroke detection during the quality improvement period, no participant had assessment using a standardized pediatric stroke scale and neuroimaging was not available.
In children with sickle cell anemia, a high rate of initial and recurrent strokes exists in a low-resource setting. Ongoing needs include training to detect strokes with an objective stroke assessment and government-supported free access to hydroxyurea for stroke prevention.
为提高尼日利亚卡诺州镰状细胞贫血儿童的护理质量，我们于 2014 年启动了一项标准护理方案，用于治疗 Aminu Kano 教学医院的镰状细胞贫血儿童中风患者。
标准护理方案要求，中风后两个月内，儿童急性中风需用羟脲治疗，剂量为 20mg/kg/天。
根据 2014 年至 2017 年的临床世界卫生组织标准，共确定了 29 名镰状细胞贫血和首发中风的儿童。中位随访时间为 1.04 年（四分位间距 0.43 至 1.83 年），直至 2017 年 7 月或第二次中风，相应的首发中风发生率为 0.88/100 患者年。8 名儿童发生复发性中风，其中 6 名在首次中风后两个月内服用了 20mg/kg/天的羟脲。有 2 名儿童死亡。6 次复发性中风发生在首次中风后 6 个月内，其中 2 次在羟脲处方之前。复发性中风发生率为 17.4/100 患者年。由于家庭必须支付羟脲费用，因此药物的依从性约为 60%。中风发病率可能被低估，因为尽管在质量改进期间进行了正式的中风检测培训，但没有参与者使用标准化的儿科中风量表进行评估，且没有神经影像学检查。
在资源匮乏的环境中，镰状细胞贫血儿童存在较高的首发和复发性中风发生率。持续的需求包括培训以使用客观的中风评估来检测中风，以及政府支持的免费获得用于预防中风的羟脲。
