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儿童风湿性疾病中的甲襞毛细血管异常。

Nailfold capillary abnormalities in childhood rheumatic diseases.

作者信息

Spencer-Green G, Schlesinger M, Bove K E, Levinson J E, Schaller J G, Hanson V, Crowe W E

出版信息

J Pediatr. 1983 Mar;102(3):341-6. doi: 10.1016/s0022-3476(83)80645-3.

Abstract

The nailfold capillary patterns of 84 patients with a variety of childhood rheumatic diseases and 34 normal control subjects were observed. Distinctive morphologic abnormalities with capillary dilation and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis and with scleroderma (P less than 0.001). Among those with scleroderma, capillary abnormalities were found in all nine patients with systemic disease and in none of 10 patients with cutaneous disease only (Fisher's exact P less than 0.001). Of 25 patients with dermatomyositis for whom muscle biopsies were available for analysis, abnormal nailfold capillary pattern was found with highest prevalence in patients with two or more specific vascular lesions noted on biopsy (Fisher's exact P = 0.041). Nailfold capillary abnormalities are present in distinct populations of childhood rheumatic diseases, reflect the underlying vasculopathy of childhood dermatomyositis, and may be of diagnostic value in distinguishing localized from systemic scleroderma.

摘要

观察了84例患有各种儿童风湿性疾病的患者以及34名正常对照者的甲襞毛细血管形态。在两组患者中发现了明显的形态学异常,伴有毛细血管扩张和周围结构缺失:儿童皮肌炎患者和硬皮病患者(P<0.001)。在硬皮病患者中,9例全身性疾病患者均发现毛细血管异常,而10例仅患有皮肤疾病的患者均未发现(Fisher精确检验P<0.001)。在25例可进行肌肉活检分析的皮肌炎患者中,活检发现有两个或更多特定血管病变的患者甲襞毛细血管形态异常的患病率最高(Fisher精确检验P = 0.041)。甲襞毛细血管异常存在于不同的儿童风湿性疾病群体中,反映了儿童皮肌炎潜在的血管病变,并且在区分局限性硬皮病和系统性硬皮病方面可能具有诊断价值。

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