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小儿结节病

Paediatric sarcoidosis.

作者信息

Fauroux Brigitte, Clément Annick

机构信息

Pediatric Pulmonology and Research Unit INSERM U719, Hôpital Armand Trousseau, 28 avenue du Docteur Arnold Netter, 75012 Paris, France.

出版信息

Paediatr Respir Rev. 2005 Jun;6(2):128-33. doi: 10.1016/j.prrv.2005.03.007.

Abstract

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, most commonly affecting young adults. Divergent prevalence rates and clinical appearances in different races indicate the existence of predisposing genes, with probably a major effect of the major histocompatibility complex. The diagnosis is relatively rare in children. Lesions can occur in almost any tissue or organ but the lungs, lymph nodes, eyes, skin and liver are the most commonly involved. Clinical symptoms are non-specific and often minor. The diagnosis can only be supported by typical histopathological findings with non-caseating epithelioid-cell granulomas. The prognosis seems to be more severe in younger children and in cases of multi-organ involvement. Corticosteroids are the therapeutic agents most commonly used and are indicated in cases of significant lung or eye lesions, cardiac, neurological, or multiorgan involvement. Close monitoring is mandatory during and after treatment because relapses are common.

摘要

结节病是一种病因不明的多系统肉芽肿性疾病,最常影响年轻人。不同种族中不同的患病率和临床表现表明存在易感基因,主要组织相容性复合体可能起主要作用。儿童中该诊断相对罕见。病变几乎可发生于任何组织或器官,但肺、淋巴结、眼、皮肤和肝脏是最常受累的部位。临床症状不具特异性且通常较轻。只有典型的组织病理学表现为非干酪样上皮样细胞肉芽肿时才能支持诊断。年幼患儿及多器官受累的病例预后似乎更差。皮质类固醇是最常用的治疗药物,适用于有明显肺部或眼部病变、心脏、神经或多器官受累的情况。治疗期间及治疗后必须密切监测,因为复发很常见。

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