青少年皮肌炎——105例皮肌炎患者的统计观察。

T Hiketa, M Ohashi
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摘要

对1965 ~ 1989年在名古屋大学医院皮肤科及爱知县部分医院就诊的105例皮肌炎(DMS)患者的临床项目进行了统计观察。通过观察获得的青少年DMS患者的临床特征如下:1) DMS幼鱼雌雄比为1.3:1。在成年DMS中没有发现明显的差异,如雌性占优势。2)观察到皮肤表现先于肌肉表现的趋势,在整个临床过程中,除严重症状外,肌肉表现的发生率较高。3)在实验室检查中,儿童血清醛缩酶浓度升高的发生率显著高于成人(p < 0.05)。血清醛缩酶浓度通常在发病时或发病前升高。因此,测定血清醛缩酶水平被认为是早期诊断青少年DMS的有用方法。儿童抗核抗体阳性率明显低于成人(p < 0.001)。4)患儿无恶性肿瘤、间质性肺炎、肺纤维化等并发症,无死亡病例。在预后方面,儿童“缓解或改善”的发生率显著高于成人(p < 0.05)。在“相同或较差”组中,与成人相比,儿童主要表现为难以治疗的皮肤表现。这些结果表明,青少年DMS可能是一种不同的疾病,可能是一种综合征,从成人DMS。
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[Juvenile dermatomyositis--statistical observation of 105 patients with dermatomyositis].

Statistical observation about the clinical items of 105 patients with dermatomyositis (DMS) attending Department of Dermatology, Nagoya University Hospital and some hospitals in Aichi Prefecture, during the year of 1965 to 1989, was carried out. Clinical features of the patients with juvenile DMS obtained from the observation were as follows. 1) The male:female ratio was 1.3:1 in juvenile DMS. The evident difference such as the predominance of females in adult DMS was not found. 2) The tendency that cutaneous manifestations usually preceded muscular manifestations was observed, and the muscular manifestations except severe symptoms were seen with a high incidence throughout the entire clinical course. 3) In the laboratory examinations, the incidence of elevation of serum aldolase concentrations in children was significantly higher than that in adults (p less than 0.05). Serum aldolase concentrations were usually elevated at onset or prior to the onset of muscular manifestations. Therefore the measurement of serum aldolase levels was considered to be useful for early diagnosis of juvenile DMS. The positive rate of antinuclear antibody in children was significantly lower than that in adults (p less than 0.001). 4) None of the children had any complications such as malignant tumors, interstitial pneumonia and pulmonary fibrosis, and none of them died. With regard to the outcome, the incidence of "remission or improvement" in children was significantly higher than that in adults (p less than 0.05). In the group of "same or worse", the children mainly had the cutaneous manifestations which were difficult to treat, compared with the adults. These results suggest that juvenile DMS may be a different disorder, probably a syndrome, from adult DMS.

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