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原发性纤毛运动障碍与真菌感染:儿童变应性支气管肺曲霉病两例。

Primary ciliary dyskinesia and fungal infections: Two cases of allergic bronchopulmonary aspergillosis in children.

机构信息

Department of Pediatrics, Division of Respirology, CHU Liège, Liège, Belgium.

Pediatric Pulmonology and Allergy Department, Hôpital Jeanne de Flandre, CHU Lille, Univ. Lille, Lille, France.

出版信息

Pediatr Pulmonol. 2022 Jul;57(7):1809-1813. doi: 10.1002/ppul.25945. Epub 2022 May 17.

Abstract

Primary ciliary dyskinesia (PCD) is a rare inherited disease that affects the movement of the respiratory cilia. The main clinical manifestations are chronic upper and lower respiratory symptoms and recurrent lung infections, particularly bacterial and viral infections. Fungal infections are not usually associated with PCD. Allergic bronchopulmonary aspergillosis (ABPA) is a rare complex immune hypersensitivity reaction to Aspergillus fumigatus reported in patients with asthma and cystic fibrosis. Only three cases of ABPA associated with adult PCD have been described in the literature. Herein, we reported on two cases of ABPA in two boys aged 10 and 13 years with PCD. Both had severe lung disease and chronic Pseudomonas aeruginosa infections. One patient was diagnosed according to the typical clinical features of ABPA, while the other was diagnosed during a scheduled visit with no clinical changes but worsening pulmonary function and radiologic anomalies. The diagnosis of ABPA was confirmed in the two patients who then improved after receiving specific treatment. These two cases were the first to describe the occurrence of ABPA in children with PCD. We recommend that physicians involved in the management of children with PCD be aware of this potential complication.

摘要

原发性纤毛运动障碍(PCD)是一种罕见的遗传性疾病,影响呼吸道纤毛的运动。主要临床表现为慢性上下呼吸道症状和复发性肺部感染,特别是细菌和病毒感染。真菌感染通常与 PCD 无关。变应性支气管肺曲霉病(ABPA)是一种罕见的复杂免疫过敏反应,据报道发生在哮喘和囊性纤维化患者的烟曲霉中。文献中仅描述了三例与成人 PCD 相关的 ABPA。在此,我们报告了两例年龄分别为 10 岁和 13 岁的 PCD 男孩的 ABPA 病例。两人均患有严重的肺部疾病和慢性铜绿假单胞菌感染。一名患者根据 ABPA 的典型临床特征进行诊断,另一名患者在无临床变化但肺功能恶化和影像学异常的情况下进行定期就诊时进行诊断。这两名患者的 ABPA 诊断得到确认,随后在接受特异性治疗后有所改善。这两例是首次描述 PCD 患儿发生 ABPA 的病例。我们建议参与儿童 PCD 管理的医生注意这种潜在的并发症。

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