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中枢神经系统获得性脱髓鞘疾病:一项儿科队列研究。

Acquired demyelinating disorders of central nervous system: A pediatric cohort.

作者信息

Gulati Sheffali, Chakrabarty Biswaroop, Kumar Atin, Jain Puneet, Patel Harsh, Saini Lokesh

机构信息

Department of Pediatrics, Jai Prakash Narayan Apex Trauma Centre, All India Institute of Medical Sciences, New Delhi, India.

Department of Radio-Diagnosis, Jai Prakash Narayan Apex Trauma Centre, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 1):S48-55. doi: 10.4103/0972-2327.164829.

Abstract

OBJECTIVE

This is a retrospective chart review of consecutive children with acquired demyelinating disorders presenting to a north Indian tertiary care hospital over 4 years. The aim of this review is to describe all the patients (with single event as well as those with recurrences) with detailed description of those who recurred.

MATERIALS AND METHODS

Overall 35 cases were reviewed and their clinical presentations, diagnosis, management, and follow-up are being presented.

RESULTS

Out of 35 cases, 24 did not show any recurrences (seven acute disseminated encephalomyelitis (ADEM) and 17 clinically isolated syndromes). Amongst the 11 patients with recurrent demyelination, majority were multiple sclerosis (8/11, 72.7%) followed by neuromyelitis optica (NMO; 2/11), and multiphasic ADEM (1/11). The median disease duration and follow-up since onset for those with recurrent episodes is 4 years (2.5-4.5 years). Steroids caused significant improvement in acute episodes of demyelination. However, recurrent demyelinating disorders like multiple sclerosis and NMO required long-term immunomodulation. Azathioprine currently is the most favored long-term immunomodulator used in NMO. Interferon-β and glatiramer acetate are currently recommended for multiple sclerosis. However, azathioprine may be a suitable alternative in a resource-limited setting.

CONCLUSION

The consensus definitions for these groups of disorders need further validation in the pediatric age group. Studies with larger population size are required to characterize features that predict future recurrences.

摘要

目的

这是一项对一家印度北部三级护理医院4年来收治的连续性获得性脱髓鞘疾病患儿的回顾性病历审查。本审查的目的是描述所有患者(包括单次发病以及复发患者),并详细描述复发患者的情况。

材料与方法

共审查了35例病例,并展示了他们的临床表现、诊断、治疗及随访情况。

结果

35例病例中,24例未出现复发(7例急性播散性脑脊髓炎(ADEM)和17例临床孤立综合征)。在11例复发脱髓鞘患者中,多数为多发性硬化(8/11,72.7%),其次是视神经脊髓炎(NMO;2/11)和多相性ADEM(1/11)。复发发作患者自发病起的疾病持续时间和随访时间中位数为4年(2.5 - 4.5年)。类固醇在脱髓鞘急性发作期有显著改善作用。然而,像多发性硬化和NMO这样的复发性脱髓鞘疾病需要长期免疫调节。硫唑嘌呤目前是NMO中最常用的长期免疫调节剂。目前推荐干扰素-β和醋酸格拉替雷用于治疗多发性硬化。然而,在资源有限的情况下,硫唑嘌呤可能是一种合适的替代药物。

结论

这些疾病组的共识定义在儿童年龄组中需要进一步验证。需要开展更大规模人群的研究来明确预测未来复发的特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66a2/4604698/cc9da8673414/AIAN-18-48-g006.jpg

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