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经皮给予组织型纤溶酶原激活剂治疗塑料支气管症。

Management of plastic bronchitis with topical tissue-type plasminogen activator.

机构信息

Division of Pulmonary Medicine, Department of Pediatrics, Benioff Children’s Hospital, University of California San Francisco, San Francisco, California 94143, USA.

出版信息

Pediatrics. 2012 Aug;130(2):e446-50. doi: 10.1542/peds.2011-2883. Epub 2012 Jul 16.

Abstract

Plastic bronchitis or cast bronchitis is a rare disease of unclear etiology characterized by formation of airway casts that can lead to life-threatening airway obstruction. There is currently limited data regarding optimal treatment of plastic bronchitis. Several therapies have been suggested, but recurrences are common and mortality remains high. We report the case of a 6-year-old boy with refractory eosinophilic bronchial casts, unresponsive to low-dose systemic corticosteroids, inhaled corticosteroids, azithromycin, and dornase alfa, who was treated successfully and safely with direct instillation of tissue-type plasminogen activator (tPA) to the obstructing casts during flexible bronchoscopy and inhaled tPA. Our case illustrates that the current therapy for plastic bronchitis remains inadequate. To our knowledge, this case is the first to show that direct instillation of tPA can be used safely for treatment of this disease. The use of tPA via direct administration into the airways during bronchoscopy and via a nebulizer appeared to be a safe and effective therapy for plastic bronchitis and should be considered early in the course of the disease to prevent complications of severe airway obstruction.

摘要

气道铸型或塑型支气管炎是一种罕见的病因不明的疾病,其特征是气道铸型的形成,可导致危及生命的气道阻塞。目前关于塑型性支气管炎的最佳治疗方法的数据有限。已经提出了几种治疗方法,但复发很常见,死亡率仍然很高。我们报告了一例 6 岁男孩难治性嗜酸性支气管铸型,对低剂量全身皮质类固醇、吸入皮质类固醇、阿奇霉素和脱氧核糖核酸酶α无反应,通过在支气管镜检查期间直接将组织型纤溶酶原激活剂(tPA)注入阻塞的铸型以及吸入 tPA 成功且安全地治疗了该患者。我们的病例表明,目前治疗塑型性支气管炎的方法仍然不够。据我们所知,该病例首次表明直接注入 tPA 可安全用于治疗该病。通过支气管镜检查直接将 tPA 注入气道以及通过雾化器给药似乎是治疗塑型性支气管炎的一种安全有效的方法,应在疾病早期考虑使用,以预防严重气道阻塞的并发症。

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