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儿童系统性硬化症。

Systemic sclerosis in childhood.

作者信息

Foeldvari I

机构信息

Senior Consultant Pediatric Rheumatology Clinic, Allgemeines Krankenhaus Eilbek, Haus 6, Friedrichsberger Str. 60, D-22081 Hamburg, Germany.

出版信息

Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii28-9. doi: 10.1093/rheumatology/kel292.

DOI:10.1093/rheumatology/kel292
PMID:16987828
Abstract

Juvenile systemic sclerosis (jSSc) is a rare disease of childhood, and the amount of published data is limited. It appears that its clinical presentation differs from adult disease and the limited form affects only very few children. The organ involvement pattern differs also from the adult form. Prognosis seems to be better with a 5-yr survival of 95% of the jSSc patients. The validation of the outcome measures for children with jSSc is currently in progress. Regarding effective treatment, there are no paediatric data and the paediatric rheumatologist needs to rely on the experiences in adult disease.

摘要

青少年系统性硬化症(jSSc)是一种罕见的儿童疾病,已发表的数据量有限。其临床表现似乎与成人疾病不同,且局限性型仅影响极少数儿童。器官受累模式也与成人型不同。jSSc患者5年生存率为95%,预后似乎较好。目前正在对jSSc患儿的疗效评估指标进行验证。关于有效治疗,尚无儿科数据,儿科风湿病学家需要依靠成人疾病的治疗经验。

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Systemic sclerosis in childhood.儿童系统性硬化症。
Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii28-9. doi: 10.1093/rheumatology/kel292.
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引用本文的文献

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Juvenile systemic sclerosis - observations of one clinical centre.青少年系统性硬化症——一个临床中心的观察
Reumatologia. 2021;59(6):367-372. doi: 10.5114/reum.2021.112350. Epub 2022 Jan 12.