Abuga Jonathan A, Kariuki Symon M, Kinyanjui Samson M, Boele van Hensbroek Michael, Newton Charles R
Kenya Medical Research Institute (KEMRI-Wellcome Trust Research Programme), Clinical Research (Neurosciences), Kilifi, Kenya.
Global Child Health Group, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands.
Front Neurol. 2021 Apr 9;12:627824. doi: 10.3389/fneur.2021.627824. eCollection 2021.
Neurological impairment (NI) and disability are associated with reduced life expectancy, but the risk and magnitude of premature mortality in children vary considerably across study settings. We conducted a systematic review to estimate the magnitude of premature mortality following childhood-onset NI worldwide and to summarize known risk factors and causes of death. We searched various databases for published studies from their inception up to 31st October 2020. We included all cohort studies that assessed the overall risk of mortality in individuals with childhood-onset epilepsy, intellectual disability (ID), and deficits in hearing, vision and motor functions. Comparative measures of mortality such as the standardized mortality ratio (SMR), risk factors and causes were synthesized quantitatively under each domain of impairment. This review is registered on the PROSPERO database (registration number CRD42019119239). The search identified 2,159 studies, of which 24 studies were included in the final synthesis. Twenty-two (91.7%) studies originated from high-income countries (HICs). The median SMR was higher for epilepsy compared with ID (7.1 [range 3.1-22.4] vs. 2.9 [range 2.0-11.6]). In epilepsy, mortality was highest among younger age groups, comorbid neurological disorders, generalized seizures (at univariable levels), untreatable epilepsy, soon after diagnosis and among cases with structural/metabolic types, but there were no differences by sex. Most deaths (87.5%) were caused by non-epilepsy-related causes. For ID, mortality was highest in younger age groups and girls had a higher risk compared to the general population. Important risk factors for premature mortality were severe-to-profound severity, congenital disorders e.g., Down Syndrome, comorbid neurological disorders and adverse pregnancy and perinatal events. Respiratory infections and comorbid neurological disorders were the leading causes of death in ID. Mortality is infrequently examined in impairments of vision, hearing and motor functions. The risk of premature mortality is elevated in individuals with childhood-onset NI, particularly in epilepsy and lower in ID, with a need for more studies for vision, hearing, and motor impairments. Survival in NI could be improved through interventions targeting modifiable risk factors and underlying causes.
神经功能障碍（NI）和残疾与预期寿命缩短有关，但儿童过早死亡的风险和程度在不同研究环境中差异很大。我们进行了一项系统综述，以估计全球儿童期发病的NI后过早死亡的程度，并总结已知的风险因素和死亡原因。我们在各个数据库中搜索了从开始到2020年10月31日发表的研究。我们纳入了所有评估儿童期发病的癫痫、智力残疾（ID）以及听力、视力和运动功能缺陷个体总体死亡风险的队列研究。在每个损伤领域下，对死亡率的比较指标如标准化死亡比（SMR）、风险因素和原因进行了定量综合分析。本综述已在PROSPERO数据库注册（注册号CRD42019119239）。搜索共识别出2159项研究，其中24项研究纳入最终综合分析。22项（91.7%）研究来自高收入国家（HICs）。与ID相比，癫痫的SMR中位数更高（7.1[范围为3.1 - 22.4]对2.9[范围为2.0 - 11.6]）。在癫痫中，死亡率在较年轻年龄组、合并神经疾病、全身性发作（单变量水平）、无法治疗的癫痫、诊断后不久以及结构性/代谢性类型的病例中最高，但性别之间无差异。大多数死亡（87.5%）由非癫痫相关原因引起。对于ID，死亡率在较年轻年龄组最高，女孩与一般人群相比风险更高。过早死亡的重要风险因素包括重度至极重度、先天性疾病（如唐氏综合征）、合并神经疾病以及不良妊娠和围产期事件。呼吸道感染和合并神经疾病是ID死亡的主要原因。视力、听力和运动功能损伤方面的死亡率很少被研究。儿童期发病的NI个体过早死亡风险升高，尤其是在癫痫中，而在ID中较低，需要对视力、听力和运动损伤进行更多研究。通过针对可改变的风险因素和潜在原因的干预措施，可以改善NI患者的生存情况。
