Mateos-Corral Dimas, Cutz Ernest, Solomon Melinda, Ratjen Felix
Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Canada.
Pediatr Pulmonol. 2009 Sep;44(9):939-40. doi: 10.1002/ppul.21063.
Cystic fibrosis patients are known to produce abundant, purulent sputum consisting mainly of DNA and cellular debris. We present a case of a CF patient with recurrent airway obstruction caused by a rare condition known as plastic bronchitis (PB). PB is characterized by the formation of casts of the airways that cause obstruction. Multiple etiologies have been proposed, but to our knowledge, no CF patient has been reported in any PB classification. Histological analysis and in-vitro testing of the cast were important factors in choosing the adequate therapy in this patient.
已知囊性纤维化患者会咳出大量主要由DNA和细胞碎片组成的脓性痰液。我们报告一例囊性纤维化患者,其因一种罕见的称为塑性支气管炎(PB)的病症而反复出现气道阻塞。塑性支气管炎的特征是形成导致阻塞的气道铸型。虽然已经提出了多种病因,但据我们所知,在任何塑性支气管炎分类中都没有报道过囊性纤维化患者。对铸型进行组织学分析和体外测试是为该患者选择适当治疗方法的重要因素。