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儿童期的假性支气管扩张症:临床表现、病因、治疗和结局的多样性。

Plastic bronchitis during childhood: Diversity of presentation, etiology, treatment, and outcomes.

机构信息

Division of Pulmonology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Division of Allergy and Asthma, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

出版信息

Pediatr Pulmonol. 2023 Sep;58(9):2559-2567. doi: 10.1002/ppul.26548. Epub 2023 Jun 6.

Abstract

OBJECTIVE

Plastic bronchitis (PB) is a rare disease in children, and reliable data are scarce. Here, we aimed to analyze the clinical features, management, and outcomes in children with PB.

METHODS

The medical data of patients who were followed up with a diagnosis of PB between January 2010 and March 2022 were retrospectively analyzed.

RESULTS

The median age of 15 patients was 9 (interquartile range: 4-10) years with a male/female ratio of 12/3. Initial symptoms included recurrent pneumonia (33.3%), persistent atelectasis (33.3%), cast expectoration (26.6%), and intense, persistent cough (6.6%). The most common underlying diagnosis was asthma (n = 12, 80%), and six of the patients were newly diagnosed. The most common radiological findings were atelectasis as a consequence of major airway obstruction on chest X-ray or computed tomography. Five patients, all diagnosed as having asthma, had recurrent PB and required multiple airway procedures for treatment and diagnosis. During a median 7-year follow-up of five patients, occasionally cast expectoration was observed in one patient with asthma who had poor compliance with inhaled corticosteroids.

CONCLUSION

PB is a common reflection of the different underlying etiologies in the pediatric age group, and treatment and outcomes are closely related to these. It should be kept in mind that asthma can be a predisposing factor for the development of PB.

摘要

目的

小儿塑料性支气管炎(PB)是一种罕见疾病,可靠数据较为缺乏。本研究旨在分析小儿 PB 患者的临床特征、处理方法和结局。

方法

回顾性分析 2010 年 1 月至 2022 年 3 月期间经随访诊断为 PB 的患者的临床资料。

结果

15 例患者的中位年龄为 9 岁(四分位距:4-10 岁),男/女比例为 12/3。首发症状包括反复肺炎(33.3%)、持续肺不张(33.3%)、痰栓(26.6%)和剧烈、持续咳嗽(6.6%)。最常见的基础诊断为哮喘(n=12,80%),其中 6 例为新诊断。最常见的影像学表现为胸部 X 线或 CT 上大气道阻塞导致的肺不张。5 例被诊断为哮喘的患者均出现复发性 PB,需要多次气道介入治疗以明确诊断和治疗。在 5 例患者的中位 7 年随访期间,1 例哮喘患儿因吸入性皮质激素治疗依从性差,偶尔出现痰栓。

结论

PB 是儿科不同基础病因的常见表现,治疗和结局与病因密切相关。应注意哮喘可能是发生 PB 的一个易感因素。

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