儿童吉兰-巴雷综合征脑脊液神经丝蛋白轻链特点分析

doi:10.12372/jcp.2024.22e1062

摘要/Abstract

摘要：

目的探讨脑脊液神经丝蛋白轻链（NfL）对儿童吉兰-巴雷综合征（GBS）患者早期诊断及预后的评估价值。方法回顾性分析2020年11月至2022年5月收治的GBS患儿的临床资料，选择同期诊断为偏头痛、儿童情绪障碍患者作为对照组，比较两组脑脊液-NfL水平，分析脑脊液-NfL水平与临床特征的相关性。结果 GBS组患儿26例，男14例、女12例，中位年龄5.0（3.8~8.0）岁；对照组48例，男30例、女18例，中位年龄8.0（5.0~9.0）岁。入院时GBS组脑脊液-NfL和脑脊液蛋白水平高于对照组，差异有统计学意义（P<0.05）。联合检测脑脊液-NfL和蛋白早期诊断GBS的灵敏度、特异度、阳性预测值、阴性预测值和准确度分别为92.3%、95.8%、92.3%、95.8%和94.6%。入院时GBS患儿脑脊液蛋白水平及疾病高峰时Hughes评分均与脑脊液-NfL水平呈显著正相关（P<0.05）。预后不良组患儿脑脊液-NfL水平显著高于预后良好组，差异有统计学意义（P<0.05）。结论脑脊液-NfL与脑脊液蛋白水平、疾病严重程度及短期预后相关，对于早期辅助诊断GBS、评估短期预后有一定帮助。

关键词: 吉兰-巴雷综合征, 神经丝蛋白轻链, Hughes功能评分, 短期预后, 儿童

Abstract:

Objective To evaluate the value of cerebrospinal fluid neurofilament light chain (CSF-NfL) levels in early diagnosis and prognosis of children with Guillain-Barre syndrome (GBS). Methods The clinical data of children with GBS admitted from November 2020 to May 2022 were retrospectively analyzed, and the children diagnosed with migraine and psychological disorders during the same period were selected as the control group. The level of CSF-NfL was compared between the two groups, and the correlation between the level of CSF-NfL and clinical characteristics was analyzed. Results There were 26 children (14 boys and 12 girls) in the GBS group, with a median age of 5.0 (3.8-8.0) years. The control group consisted of 48 children (30 boys and 18 girls) with a median age of 8.0 (5.0-9.0) years. At admission, the levels of CSF-NfL and CSF protein in the GBS group were higher than those in the control group, and the difference was statistically significant (P<0.05). The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of CSF-NfL combined with CSF protein levels in the early diagnosis of GBS were 92.3%, 95.8%, 92.3%, 95.8% and 94.6%, respectively. The CSF protein level at admission and Hughes functional score at the peak of disease were significantly positively correlated with CSF-NfL level in children with GBS (P<0.05). The CSF-NfL levels of GBS patients with poor prognosis were significantly higher than those of patients with good prognosis, and the difference was statistically significant (P<0.05). Conclusions The CSF-NfL levels were associated with CSF protein levels, disease severity and short-term prognosis, which may be helpful for the early diagnosis and prognosis evaluation of GBS.

Key words: Guillain-Barré syndrome, neurofilament light chain, Hughes functional score, short-term prognosis, child

靳梅, 刘静, 刘康, 赵力搏, 赵紫薇, 孙素真. 儿童吉兰-巴雷综合征脑脊液神经丝蛋白轻链特点分析[J]. 临床儿科杂志, 2024, 42(1): 58-62.

JIN Mei, LIU Jing, LIU Kang, ZHAO Libo, ZHAO Ziwei, SUN Suzhen. Characteristics of neurofilament light chain levels in cerebrospinal fluid of Guillain-Barre syndrome in children[J]. Journal of Clinical Pediatrics, 2024, 42(1): 58-62.

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组别	例数	年龄[M(P₂₅~P₇₅)]/岁	性别(男/女)	脑脊液-NfL[M(P₂₅~P₇₅)]/pg·mL^-1	脑脊液蛋白[M(P₂₅~P₇₅)]/g·L^-1
GBS组	26	5.0(3.8~8.0)	14/12	108.9(97.4~124.7)	0.9(0.7~1.3)
对照组	48	8.0(5.0~9.0)	30/18	80.0(67.9~87.4)	0.3(0.2~0.3)
统计量		Z=1.54	χ²=0.52	Z=5.38	Z=5.55
P		0.123	0.469	<0.001	<0.001

因素	r_s值	P
年龄	0.06	0.762
起病至疾病高峰时间	0.25	0.220
疾病高峰时Hughes评分	0.46	0.018
脑脊液蛋白	0.39	0.048
脑脊液细胞数	0.12	0.546

因素	例数	CSF-NfL/pg·mL^-1	统计量	P
性别(x±s)			t=1.48	0.152
男	14	118.7±29.7
女	12	103.7±20.1
前驱感染史(呼吸道或胃肠道)(x±s)			t=0.65	0.523
有	17	114.2±26.8
无	9	107.1±26.3
神经系统症状(面神经或球麻痹)[M(P₂₅~P₇₅)]			Z=0.84	0.403
有	9	120.1(99.3~128.2)
无	17	107.4(95.2~126.1)
自主神经功能障碍(x±s)			t=1.08	0.292
有	19	115.1±26.8
无	7	102.6±24.6
机械通气(x±s)			t=0.11	0.911
有	5	110.5±20.9
无	21	112.1±27.9
电生理亚型[M(P₂₅~P₇₅)]			Z=1.41	0.160
AIDP	23	110.3(97.8~135.1)
AMAN	3	101.8(91.4~102.0)
治疗[M(P₂₅~P₇₅)]			Z=0.44	0.659
IVIg	23	108.6(97.8~121.3)
IVIg+血浆置换	3	120.1(91.4~142.5)
短期预后(x±s)
良好	19	104.9±24.2	t=2.40	0.025
不良	7	130.5±24.0