Division of Pulmonary Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Pediatr Pulmonol. 2018 Nov;53(11):1574-1581. doi: 10.1002/ppul.24161. Epub 2018 Sep 12.
Percutaneous lymphatic intervention (PCL) is a promising new therapy for plastic bronchitis (PB). We characterized bronchoalveolar lavage (BAL) and cast morphology in surgically repaired congenital heart disease (CHD) patients with PB during PCL. We quantified respiratory and bronchoscopic characteristics and correlated them with post-intervention respiratory outcomes.
We retrospectively reviewed patients with PB and surgically repaired CHD undergoing PCL and bronchoscopy at our institution. Pre-intervention characteristics, bronchoscopy notes, BAL cell counts, virology, and cultures were collected. A pathologist blinded to clinical data reviewed cast specimens. Respiratory outcomes were evaluated through standardized telephone questionnaire.
Sixty-two patients were included with a median follow-up of 20 months. No patients experienced airway bleeding, obstruction, or prolonged intubation related to bronchoscopy. Of BAL infectious studies, the positive results were 4 (8%) fungal, 6 (11%) bacterial, and 6 (14%) viral. Median BAL count per 100 cells for neutrophils, lymphocytes, and eosinophils were 13, 10, and 0, respectively. Of 23 bronchial casts analyzed, all contained lymphocytes, and 19 (83%) were proteinaceous, with 14 containing neutrophils and/or eosinophils. Median BAL neutrophil count was greater in patients with proteinaceous neutrophilic or eosinophilic casts compared to casts without neutrophils or lymphocytes (P = 0.030). Post-intervention, there was a significant reduction in respiratory medications and support and casting frequency.
The predominance of neutrophilic proteinaceous casts and high percentage of positive BAL infectious studies support short-term fibrinolytic and anti-infective therapies in PB in select patients. Flexible bronchoscopy enables safe assessment of cast burden. PCL effectively treats PB and reduces respiratory therapies.
经皮淋巴管介入(PCL)是治疗塑料性支气管炎(PB)的一种有前途的新疗法。我们在经皮淋巴管介入(PCL)期间对接受 PB 治疗的接受过心脏先天性疾病(CHD)手术修复的患者的支气管肺泡灌洗(BAL)和铸型形态进行了特征描述。我们对呼吸和支气管镜特征进行了量化,并将其与介入后的呼吸结果进行了相关性分析。
我们回顾性地审查了在我们机构接受 PB 和心脏先天性疾病(CHD)手术修复并接受 PCL 和支气管镜检查的患者。收集了介入前特征、支气管镜检查记录、BAL 细胞计数、病毒学和培养物。一位对临床数据不知情的病理学家审查了铸型标本。通过标准化电话问卷评估了呼吸结果。
共纳入 62 例患者,中位随访时间为 20 个月。没有患者因支气管镜检查而出现气道出血、阻塞或长时间插管。在 BAL 传染性研究中,阳性结果为 4(8%)例真菌、6(11%)例细菌和 6(14%)例病毒。100 个细胞中 BAL 中性粒细胞、淋巴细胞和嗜酸性粒细胞的中位数分别为 13、10 和 0。在分析的 23 个支气管铸型中,所有铸型均含有淋巴细胞,19(83%)为蛋白性,其中 14 个含有中性粒细胞和/或嗜酸性粒细胞。与无中性粒细胞或淋巴细胞的铸型相比,含蛋白性中性粒细胞或嗜酸性粒细胞的铸型患者的 BAL 中性粒细胞计数更高(P=0.030)。介入后,呼吸药物和支持治疗以及铸型形成的频率显著减少。
在某些患者中,以中性粒细胞蛋白性铸型为主,BAL 传染性研究阳性率高,支持短期纤维蛋白溶解和抗感染治疗。纤维支气管镜可安全评估铸型负荷。PCL 可有效治疗 PB 并减少呼吸治疗。