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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.

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/1702/9955750/197ea645ad24/children-10-00262-g001.jpg

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