Aurélie Sarthou, Pascale Chrétien, Laetitia Giorgi, Andrada Chiron, Carole Leroy, Philippe Horellou, Roman Krzysiek, Kumaran Deiva, Salima Hacein-Bey-Abina
{"title":"卡帕游离轻链指数是儿科多发性硬化症的准确诊断生物标志物。","authors":"Aurélie Sarthou, Pascale Chrétien, Laetitia Giorgi, Andrada Chiron, Carole Leroy, Philippe Horellou, Roman Krzysiek, Kumaran Deiva, Salima Hacein-Bey-Abina","doi":"10.1177/13524585241274034","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Objective: </strong>To assess the diagnostic value of the KFLC index in a cohort of patients with paediatric-onset, inflammatory disorders of the CNS.</p><p><strong>Methods: </strong>We included 73 patients and divided them into four groups: PedMS (<i>n</i> = 16), ODS (<i>n</i> = 17), encephalitis and/or inflammatory epilepsy (EE, <i>n</i> = 15), and controls without inflammatory CNS diseases (<i>n</i> = 25). The KFLC index was calculated and compared with the results of the oligoclonal bands determination.</p><p><strong>Results: </strong>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%).</p><p><strong>Conclusion: </strong>The KFLC index is a reliable tool for the diagnosis of MS in a paediatric population.</p>","PeriodicalId":18874,"journal":{"name":"Multiple Sclerosis Journal","volume":null,"pages":null},"PeriodicalIF":4.8000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The kappa free light chains index is an accurate diagnostic biomarker for paediatric multiple sclerosis.\",\"authors\":\"Aurélie Sarthou, Pascale Chrétien, Laetitia Giorgi, Andrada Chiron, Carole Leroy, Philippe Horellou, Roman Krzysiek, Kumaran Deiva, Salima Hacein-Bey-Abina\",\"doi\":\"10.1177/13524585241274034\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Objective: </strong>To assess the diagnostic value of the KFLC index in a cohort of patients with paediatric-onset, inflammatory disorders of the CNS.</p><p><strong>Methods: </strong>We included 73 patients and divided them into four groups: PedMS (<i>n</i> = 16), ODS (<i>n</i> = 17), encephalitis and/or inflammatory epilepsy (EE, <i>n</i> = 15), and controls without inflammatory CNS diseases (<i>n</i> = 25). The KFLC index was calculated and compared with the results of the oligoclonal bands determination.</p><p><strong>Results: </strong>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%).</p><p><strong>Conclusion: </strong>The KFLC index is a reliable tool for the diagnosis of MS in a paediatric population.</p>\",\"PeriodicalId\":18874,\"journal\":{\"name\":\"Multiple Sclerosis Journal\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":4.8000,\"publicationDate\":\"2024-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Multiple Sclerosis Journal\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1177/13524585241274034\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/9/8 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Multiple Sclerosis Journal","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/13524585241274034","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/9/8 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
The kappa free light chains index is an accurate diagnostic biomarker for paediatric multiple sclerosis.
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 (n = 16), ODS (n = 17), encephalitis and/or inflammatory epilepsy (EE, n = 15), and controls without inflammatory CNS diseases (n = 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.
期刊介绍：
Multiple Sclerosis Journal is a peer-reviewed international journal that focuses on all aspects of multiple sclerosis, neuromyelitis optica and other related autoimmune diseases of the central nervous system.
The journal for your research in the following areas:
* __Biologic basis:__ pathology, myelin biology, pathophysiology of the blood/brain barrier, axo-glial pathobiology, remyelination, virology and microbiome, immunology, proteomics
* __Epidemology and genetics:__ genetics epigenetics, epidemiology
* __Clinical and Neuroimaging:__ clinical neurology, biomarkers, neuroimaging and clinical outcome measures
* __Therapeutics and rehabilitation:__ therapeutics, rehabilitation, psychology, neuroplasticity, neuroprotection, and systematic management
Print ISSN: 1352-4585
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