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以儿童风湿性表现为特征的埃勒斯-当洛综合征。

Ehlers-Danlos syndrome presenting as rheumatic manifestations in the child.

作者信息

Osborn T G, Lichtenstein J R, Moore T L, Weiss T, Zuckner J

出版信息

J Rheumatol. 1981 Jan-Feb;8(1):79-85.

PMID:7218262
Abstract

Nine patients with Ehlers-Danlos syndrome (EDS) presented with joint complaints. This represented 5% of the new pediatric arthritis patients seen over a 6-month period. All 9 patients had a history of double-jointedness, and 7 had arthralgias associated with increased physical activity. On physical examination all had hypermobile joints, 7 had joint tenderness, but none had joint swelling. Based on the presence of other features including hyperextensible skin, widened scars, and cardiac clicks and murmurs, 8 were categorized as EDS Type II and 1 as EDS Type III. It posed a significant problem because the diagnosis had not been previously entertained before consultation and erroneous diagnosis had been made. Therefore, EDS should be included in the differential diagnosis of polyarthralgia in the child.

摘要

9例埃勒斯-当洛综合征(EDS)患者出现关节问题。这占6个月内新诊断的小儿关节炎患者的5%。所有9例患者都有双关节病史,7例有关节痛且与体力活动增加有关。体格检查时,所有患者关节活动过度,7例有关节压痛,但均无关节肿胀。基于其他特征,包括皮肤过度伸展、瘢痕增宽以及心脏喀喇音和杂音,8例被归类为Ⅱ型EDS,1例为Ⅲ型EDS。这带来了一个重大问题,因为在会诊前未考虑到该诊断,且已做出错误诊断。因此,小儿多关节痛的鉴别诊断应包括EDS。

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