摘要
儿童结节病是一种罕见的不明病因的全身性疾病,其特征在于一个或多个器官及系统的非干酪性肉芽肿性炎症。肺结节病主要临床表现为肺部浸润,肺门淋巴结大,伴或不伴眼部及皮肤病变。儿童肺结节病临床表现多样且非特异,甚至部分结节病患儿无症状;尽管影像学,血清生物标志物,介入肺病学和遗传学方面的进展已经扩大了临床医师对肺结节病的认识,但该病的识别与诊断仍是一大挑战。目前儿童结节病主要采用以口服激素为主,必要时辅以甲氨蝶呤的免疫抑制方法治疗,预后较成人更佳。越来越多的研究为结节病的诊断及治疗积累了经验、方法及证据。
Childhood sarcoidosis is a rare systemic disease of unknown etiology,characterized by the noncaseating granulomatous inflammation of one or more organs/systems. The clinical presentations are pulmonary infiltration,hilar lymphadenopathy,with/without ocular or cutaneous lesions. The clinical manifestations are diversified and non-specific,and some children with sarcoidosis are asymptomatic. Although advances in imaging,biomarkers,interventional pulmonology and genetics have increasingly broadened the knowledge of the disease,the identification and diagnosis of childhood sarcoidosis is still a huge challenge to the clinicians. Currently,the disease is mainly treated by oral hormone,supplemented by methotrexate and other immunosuppressive agents if necessary,and the prognosis is better than that of the adults. More and more studies have accumulated experiences,methods and evidence for the diagnosis and treatment of sarcoidosis.
作者
代继宏
蹇惠
DAI Jihong;JIAN Hui(Center of Respiratory Disorders,Children′s Hospital,Chongqing Medical University,Chongqing 400014,China)
出处
《医学综述》
2018年第14期2727-2732,共6页
Medical Recapitulate
关键词
儿童结节病
临床表现
诊断
治疗
Childhood sarcoidosis
Clinical manifestation
Diagnosis
Treatment
