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喉气管狭窄需紧急气管切开术作为儿童复发性多软骨炎的首发表现。

Laryngotracheal stenosis requiring emergency tracheostomy as the first manifestation of childhood-relapsing polychondritis.

作者信息

Buscatti Izabel M, Giacomin Maria Fernanda A, Silva Marco Felipe C, Campos Lúcia M A, Sallum Adriana M E, Silva Clovis A

出版信息

Acta Reumatol Port. 2013 Jul-Sep;38(3):208-11.

Abstract

Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory, recurrent and destructive cartilage lesions. The chondritis could be widespread and involves generally laryngeal and auricular hyaline cartilages. We described a 9 years and 4 months old girl, who presented recurrent acute laryngotracheitis and laryngotracheal stenosis, which were the first manifestations of relapsing polychondritis, and was submitted to emergency tracheostomy. She also had ear condritis and arthritis, being treated with prednisolone and methotrexate. In conclusion, we reported a rare case of relapsing polychondritis that presented a life-threatening laryngo-tracheo-bronchial disorder requiring tracheostomy. We suggest that the diagnosis of relapsing polychondritis should be considered for patients who present recurrent acute laryngotracheitis with other types of condritis, as well as musculoskeletal manifestations.

摘要

复发性多软骨炎是一种病因不明的罕见儿童疾病,其特征为炎症性、复发性和破坏性软骨病变。软骨炎可能广泛存在,通常累及喉和耳的透明软骨。我们描述了一名9岁4个月大的女孩,她出现复发性急性喉气管炎和喉气管狭窄,这是复发性多软骨炎的首发表现,并接受了紧急气管切开术。她还患有耳部软骨炎和关节炎,接受泼尼松龙和甲氨蝶呤治疗。总之,我们报告了一例罕见的复发性多软骨炎病例,该病例表现为危及生命的喉气管支气管疾病,需要进行气管切开术。我们建议,对于出现复发性急性喉气管炎并伴有其他类型软骨炎以及肌肉骨骼表现的患者,应考虑复发性多软骨炎的诊断。

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