PDF(Pubmed)
Abstract：
Esophageal atresia (EA) is a rare birth defect in which respiratory tract disorders are a major cause of morbidity. It remains unclear whether respiratory tract disorders are in part caused by alterations in airway epithelial cell functions such as the activity of motile cilia. This can be studied using airway epithelial cell culture models of patients with EA. Therefore, the aim of this study was to evaluate the feasibility to culture and functionally characterize motile cilia function in the differentiated air-liquid interface cultured airway epithelial cells and 3D organoids derived from nasal brushings and bronchoalveolar lavage (BAL) fluid from children with EA. We demonstrate the feasibility of culturing differentiated airway epithelia and organoids of nasal brushings and BAL fluid of children with EA, which display normal motile cilia function. EA patient-derived airway epithelial cultures can be further used to examine whether alterations in epithelial functions contribute to respiratory disorders in EA.
摘要：
食管闭锁（EA）是一种罕见的出生缺陷，其中呼吸道疾病是发病的主要原因。尚不清楚呼吸道疾病是否部分是由气道上皮细胞功能的改变引起的，例如活动纤毛的活性。这可以使用EA患者的气道上皮细胞培养模型进行研究。因此,这项研究的目的是评估在分化的气-液界面培养的气道上皮细胞和来自EA儿童的鼻刷和支气管肺泡灌洗（BAL）液中的3D器官中培养和功能表征活动纤毛功能的可行性。我们证明了培养EA患儿鼻刷和BAL液的分化气道上皮和类器官的可行性，显示正常活动的纤毛功能。EA患者来源的气道上皮培养物可以进一步用于检查上皮功能的改变是否导致EA中的呼吸系统疾病。
