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κ 游离轻链指数是儿科多发性硬化症的一种准确的诊断生物标志物。

The kappa free light chains index is an accurate diagnostic biomarker for paediatric multiple sclerosis.

机构信息

Clinical Immunology Laboratory, Groupe Hospitalier Universitaire Paris Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

Université Paris Cité, Unité des Technologies Chimiques et Biologiques pour la Santé, CNRS, INSERM, Paris, France.

出版信息

Mult Scler. 2024 Oct;30(11-12):1436-1444. doi: 10.1177/13524585241274034. Epub 2024 Sep 8.

Abstract

BACKGROUND

Multiple sclerosis (MS) may occur before the age of 18. Differentiation between paediatric MS (PedMS) and other demyelinating syndromes (ODSs) is challenging. In adult with MS, the kappa free light chain (KFLC) index has proven to be a reliable marker of intrathecal Ig synthesis.

OBJECTIVE

To assess the diagnostic value of the KFLC index in a cohort of patients with paediatric-onset, inflammatory disorders of the CNS.

METHODS

We included 73 patients and divided them into four groups: PedMS ( = 16), ODS ( = 17), encephalitis and/or inflammatory epilepsy (EE, = 15), and controls without inflammatory CNS diseases ( = 25). The KFLC index was calculated and compared with the results of the oligoclonal bands determination.

RESULTS

The KFLC index was higher in the PedMS group (median (interquartile range (IQR)): 150.9 (41.02-310.6)) than in the ODS (3.37 (2.22-8.11)), the EE (5.53 (2.31-25.81)) and the control group (3.41 (2.27-5.08)), respectively. The best KFLC index cut-off for differentiating between patients with PedMS and controls was 6.83 (sensitivity: 100%; specificity: 92%). A KFLC index over 93.77 indicated that the patient is very likely to have PedMS (sensitivity: 68%; specificity: 100%).

CONCLUSION

The KFLC index is a reliable tool for the diagnosis of MS in a paediatric population.

摘要

背景

多发性硬化症(MS)可能发生在 18 岁之前。区分儿科多发性硬化症(PedMS)和其他脱髓鞘综合征(ODS)具有挑战性。在成人 MS 中,κ 游离轻链(KFLC)指数已被证明是鞘内 Ig 合成的可靠标志物。

目的

评估 KFLC 指数在一组儿科发病、中枢神经系统炎症性疾病患者中的诊断价值。

方法

我们纳入了 73 名患者,并将他们分为四组:PedMS(n=16)、ODS(n=17)、脑炎和/或炎症性癫痫(EE,n=15)和无炎症性中枢神经系统疾病的对照组(n=25)。计算了 KFLC 指数,并与寡克隆带测定结果进行了比较。

结果

与 ODS(中位数(四分位距(IQR)):3.37(2.22-8.11))、EE(5.53(2.31-25.81))和对照组(3.41(2.27-5.08))相比,PedMS 组的 KFLC 指数更高(中位数(IQR):150.9(41.02-310.6))。区分 PedMS 患者和对照组的最佳 KFLC 指数截断值为 6.83(敏感性:100%;特异性:92%)。KFLC 指数超过 93.77 表明患者极有可能患有 PedMS(敏感性:68%;特异性:100%)。

结论

KFLC 指数是儿科人群 MS 诊断的可靠工具。

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