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小儿多发性硬化症——流行病学、诊断和治疗的现状。

Pediatric multiple sclerosis - current status of epidemiology, diagnosis and treatment.

机构信息

Collegium Medicum, Jan Kochanowski University in Kielce.

Department of Developmental Neurology, Poznan University of Medical Sciences, Poznań, Poland.

出版信息

Neurol Neurochir Pol. 2020;54(6):508-517. doi: 10.5603/PJNNS.a2020.0069. Epub 2020 Sep 17.

Abstract

Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disease of the central nervous system that mostly affects younger adults. However, the first symptoms of MS can appear in children and adolescents before the age of 18, and we call this paediatric MS (PMS). It is estimated that paediatric MS accounts for 3-5% of the general population of patients with MS. Despite the fundamental si-milarities to adult MS, PMS has many distinctive features. Paediatric MS has a milder course compared to adults, but leads to sig-nificant disability at an early age. PMS is relapsing-remitting in 95-98% of cases; the primary progressive manifestation is much less common than in adults. The differential diagnosis of MS in children should include other childhood demyelinating diseases, mitochondrial and metabolic diseases, connective tissue diseases, and neuroborreliosis. Differentiating acute disseminated en-cephalomyelitis (ADEM) from the first onset of MS remains the biggest challenge. Over the past 10 years, understanding of the epidemiology, pathophysiology, diagnosis, and treatment of MS in children has significantly expanded. The diagnostic criteria leading to earlier diagnosis and initiation of disease-modifying therapy (DMT) have changed, and the number of drugs used in children has increased. However, many important issues require further research. This review discusses the current state of knowledge regarding the epidemiology, diagnosis, and treatment of multiple sclerosis in children.

摘要

多发性硬化症(MS)是一种中枢神经系统的慢性炎症性脱髓鞘疾病,主要影响年轻成年人。然而,MS 的最初症状可能出现在 18 岁以下的儿童和青少年中,我们称之为小儿多发性硬化症(PMS)。据估计,小儿 MS 占 MS 总患者人群的 3-5%。尽管与成人 MS 有基本相似之处,但 PMS 具有许多独特的特征。与成年人相比,小儿 MS 的病程较轻,但在早期导致严重残疾。95-98%的病例为复发缓解型;原发性进行性表现比成年人少见得多。儿童 MS 的鉴别诊断应包括其他儿童脱髓鞘疾病、线粒体和代谢疾病、结缔组织疾病和神经伯氏疏螺旋体病。将急性播散性脑脊髓炎(ADEM)与 MS 的首次发作区分开来仍然是最大的挑战。在过去的 10 年中,人们对儿童 MS 的流行病学、病理生理学、诊断和治疗的认识有了显著的扩展。导致更早诊断和开始疾病修饰治疗(DMT)的诊断标准已经改变,儿童使用的药物数量也有所增加。然而,许多重要的问题仍需要进一步研究。这篇综述讨论了目前关于儿童多发性硬化症的流行病学、诊断和治疗的知识状况。

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