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单相髓鞘少突胶质细胞糖蛋白相关疾病、多发性硬化症和其他获得性脱髓鞘综合征患儿血清神经丝轻链水平。

Serum neurofilament light-chain levels in children with monophasic myelin oligodendrocyte glycoprotein-associated disease, multiple sclerosis, and other acquired demyelinating syndrome.

机构信息

Department of Pediatrics, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.

Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany.

出版信息

Mult Scler. 2022 Sep;28(10):1553-1561. doi: 10.1177/13524585221081090. Epub 2022 Mar 14.

Abstract

OBJECTIVE

To assess the diagnostic and prognostic potential of serum neurofilament light chain (sNfL) in children with first acquired demyelinating syndrome (ADS).

METHODS

We selected 129 children with first ADS including 19 children with myelin oligodendrocyte glycoprotein (MOG)-antibody associated disease (MOGAD), 36 MOG/AQP4-seronegative ADS, and 74 with multiple sclerosis (MS) from the BIOMARKER study cohort. All children had a complete set of clinical, radiological, laboratory data and serum for NfL measurement using a highly sensitive digital ELISA (SIMOA). A control group of 35 children with non-inflammatory neurological diseases was included. sNfL levels were compared across patient groups according to clinical, laboratory, neuroradiological features and outcome after 2 years.

RESULTS

sNfL levels were significantly increased in MOGAD, seronegative ADS and MS compared to controls (-value < 0.001), in particular in children with an acute disseminated encephalomyelitis (ADEM)-like magnetic resonance imaging (MRI) pattern ( < 0.001) or longitudinally extensive myelitis ( < 0.01). In pediatric MS, elevated sNfL levels were significantly associated with higher numbers of cerebral ( < 0.001) and presence of spinal ( < 0.05) MRI lesions at baseline and predicted a higher number of relapses ( < 0.05).

CONCLUSION

sNfL levels are significantly elevated in all three studied pediatric ADS subtypes indicating neuroaxonal injury. In pediatric MS high levels of sNfL are associated with risk factors for disease progression.

摘要

目的

评估血清神经丝轻链(sNfL)在首次获得性脱髓鞘综合征(ADS)患儿中的诊断和预后潜力。

方法

我们从 BIOMARKER 研究队列中选择了 129 名首次发生 ADS 的儿童,包括 19 名髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)患儿、36 名 MOG/AQP4 抗体阴性 ADS 患儿和 74 名多发性硬化症(MS)患儿。所有患儿均具有完整的临床、影像学、实验室数据,并采用高敏数字 ELISA(SIMOA)检测血清 NfL。纳入了 35 名患有非炎症性神经疾病的对照组患儿。根据临床、实验室、神经影像学特征和 2 年后的结局,比较各组患儿的 sNfL 水平。

结果

MOGAD、血清阴性 ADS 和 MS 患儿的 sNfL 水平均显著高于对照组(-值 < 0.001),尤其是急性播散性脑脊髓炎(ADEM)样磁共振成像(MRI)模式( < 0.001)或纵向广泛脊髓炎( < 0.01)患儿。在儿科 MS 中,sNfL 水平升高与基线时大脑( < 0.001)和脊髓( < 0.05)MRI 病变数量较多显著相关,并预测复发次数较多( < 0.05)。

结论

三种研究的儿科 ADS 亚型中 sNfL 水平均显著升高,表明存在神经轴突损伤。在儿科 MS 中,sNfL 水平升高与疾病进展的危险因素相关。

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