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Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

机译:发育期混合性结缔组织病的临床特征和结局-来自一个中心的观察性研究

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摘要

Mixed connective tissue disease is a rare systemic connective tissue disease of developmental age and it includes the features of arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus and systemic sclerosis, with presence of anti-ribonucleoprotein antibodies (anti-RNP) in serum. Early diagnosis of the disease is difficult but essential in preventing development of systemic complications, which are often irreversible. International literature does not report many studies on large cohorts of children with this disease. The aim of this retrospective study was to define clinical characteristics and long-term results of treatment of the disease in 60 children with mixed connective tissue disease hospitalized in the period between 1978 and 2018. The diagnosis was established on the basis of Kasukawa’s criteria.
机译:混合性结缔组织病是一种罕见的发育年龄的系统性结缔组织病,它具有关节炎,多发性肌炎/皮肌炎,系统性红斑狼疮和系统性硬化的特征,血清中存在抗核糖核酸抗体(anti-RNP)。疾病的早​​期诊断是困难的,但对于预防通常不可逆的全身性并发症的发展至关重要。国际文献没有报道许多有关这种疾病的大批儿童的研究。这项回顾性研究的目的是确定1978年至2018年期间住院的60例混合性结缔组织病患儿的临床特征和该病的长期治疗结果。诊断是根据Kasukawa的标准进行的。

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