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风湿性舞蹈病的皮质类固醇治疗:一家三级转诊中心27年的经验

Corticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience.

作者信息

Cappellari Alberto Maria, Rogani Greta, Filocamo Giovanni, Petaccia Antonella

机构信息

Department of Neuroscience, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Department of Pediatrics, Università degli Studi di Milano, 20122 Milan, Italy.

出版信息

Children (Basel). 2023 Jan 31;10(2):262. doi: 10.3390/children10020262.

DOI:10.3390/children10020262
PMID:36832391
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9955750/
Abstract

OBJECTIVE

The purpose of this study was to investigate the effectiveness of corticosteroid therapy for children suffering from Sydenham chorea (SC).

METHODS

The design of the study was observational, retrospective and conducted at the single center of the Rheumatology Unit of Policlinic Hospital of Milan, Italy, from May 1995 to May 2022. All data about the patients were collected from medical records.

RESULTS

From a total of 59 patients enrolled in the study (44 females and 15 males; median age 9.3 years, range 7.4-10.6 years), 49 were eligible for primary outcome analysis (10 patients were excluded due to incomplete data). Overall, 75% of patients received steroid therapy, while the remaining cases were treated with symptomatic drugs, including neuroleptics and antiseizure drugs. We found that the duration of chorea was significantly shorter in patients treated with corticosteroids in comparison to those receiving symptomatic treatment (median time: 31 vs. 41 days, = 0.023). Additionally, patients with arthritis at the onset of the disease had a longer duration of chorea than those without arthritis (median time 90.5 vs. 39 days, = 0.02). We also found that chorea recurred in 12% of the patients and seemed to be linked to a younger age at onset ( = 0.01).

CONCLUSIONS

The study suggests that corticosteroid therapy can lead to a faster resolution of SC when compared to neuroleptics and antiseizure drugs treatment.

摘要

目的

本研究旨在调查皮质类固醇疗法对患有 Sydenham 舞蹈病(SC)儿童的有效性。

方法

本研究采用观察性、回顾性设计,于1995年5月至2022年5月在意大利米兰综合医院风湿病科单中心进行。所有患者数据均从病历中收集。

结果

在总共纳入研究的59例患者中(44例女性和15例男性;中位年龄9.3岁,范围7.4 - 10.6岁),49例符合主要结局分析标准(10例因数据不完整被排除)。总体而言,75%的患者接受了类固醇治疗,其余病例采用对症药物治疗,包括抗精神病药和抗癫痫药。我们发现,与接受对症治疗的患者相比,接受皮质类固醇治疗的患者舞蹈病持续时间明显更短(中位时间:31天对41天,P = 0.023)。此外,疾病发作时有关节炎的患者舞蹈病持续时间比无关节炎的患者更长(中位时间90.5天对39天,P = 0.02)。我们还发现12%的患者舞蹈病复发,且似乎与发病时年龄较小有关(P = 0.01)。

结论

该研究表明,与抗精神病药和抗癫痫药治疗相比,皮质类固醇疗法可使SC更快得到缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/c5048b0c6a42/children-10-00262-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/197ea645ad24/children-10-00262-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/6b100725eaac/children-10-00262-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/c5048b0c6a42/children-10-00262-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/197ea645ad24/children-10-00262-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/6b100725eaac/children-10-00262-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1702/9955750/c5048b0c6a42/children-10-00262-g003.jpg

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本文引用的文献

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Sydenham's chorea: A diagnosis not to miss.西德纳姆舞蹈病:不容错过的诊断。
Rev Neurol (Paris). 2022 Oct;178(8):855-856. doi: 10.1016/j.neurol.2022.04.003. Epub 2022 Sep 20.
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SciPy 1.0: fundamental algorithms for scientific computing in Python.SciPy 1.0:Python 中的科学计算基础算法。
Nat Methods. 2020 Mar;17(3):261-272. doi: 10.1038/s41592-019-0686-2. Epub 2020 Feb 3.
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Preliminary data on prednisone effectiveness in children with Sydenham chorea.儿童抽动秽语综合征应用泼尼松治疗的初步疗效数据。
Eur J Pediatr. 2020 Jun;179(6):993-997. doi: 10.1007/s00431-020-03574-y. Epub 2020 Jan 21.
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Treatment of Chorea in Childhood.儿童舞蹈病的治疗。
Pediatr Neurol. 2020 Jan;102:10-19. doi: 10.1016/j.pediatrneurol.2019.08.013. Epub 2019 Sep 7.
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Autoimmune choreas.自身免疫性舞蹈病。
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Predictors of recurrence in Sydenham's chorea: Clinical observation from a single center.Sydenham舞蹈病复发的预测因素:来自单一中心的临床观察
Brain Dev. 2016 Oct;38(9):827-34. doi: 10.1016/j.braindev.2016.04.010. Epub 2016 May 18.
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An update on the treatment of Sydenham's chorea: the evidence for established and evolving interventions.关于 Sydenham 舞蹈病治疗的最新进展:既定和新兴干预措施的证据。
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Sydenham's chorea: a clinical follow-up of 65 patients.西德纳姆舞蹈病:65例患者的临床随访
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