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儿童肺泡蛋白沉积症的治疗性双侧肺灌洗。

Therapeutic bilateral lung lavage in a child with pulmonary alveolar proteinosis.

机构信息

Respiratory Care Department, Seattle Children's Hospital, Seattle, WA, USA.

出版信息

Pediatr Crit Care Med. 2010 May;11(3):e28-31. doi: 10.1097/PCC.0b013e3181b809d6.

DOI:10.1097/PCC.0b013e3181b809d6
PMID:20453607
Abstract

OBJECTIVE

Pulmonary alveolar proteinosis is a rare condition that can cause life-threatening respiratory failure attributable to excessive alveolar accumulation of surfactant proteins. The standard treatment for removing these secretions is through therapeutic bilateral lung lavage. Descriptions of procedures for performing therapeutic bilateral lung lavage and methods used to evaluate the overall effectiveness of this invasive procedure in children with pulmonary alveolar proteinosis have not been adequately described in the medical literature. We successfully and safely performed therapeutic bilateral lung lavage and obtained lung mechanics measurements in a child with pulmonary alveolar proteinosis.

DESIGN

Case report.

SETTING

Operating room within a pediatric hospital.

PATIENTS/SUBJECTS: A 13-yr-old boy with pulmonary alveolar proteinosis requiring serial therapeutic bilateral lung lavage for recurrent respiratory distress.

INTERVENTIONS

The patient presented to the hospital operating room for therapeutic lung lavage after a recent history of progressive dyspnea, respiratory distress, declining lung function measurements, and worsening radiographic abnormalities. We obtained baseline spirometric and respiratory system compliance measurements before and after therapeutic bilateral lung lavage. The left lung was lavaged on the first day and the right lung was lavaged on the third day using selective endobronchial intubation and selective lung ventilation.

RESULTS

The procedure was well-tolerated and resulted in the removal of a significant volume of accumulated secretions. After the lavage, the patient demonstrated improvement in respiratory distress, chest radiograph appearance, lung compliance, and spirometric measurements.

CONCLUSIONS

This case report demonstrates that therapeutic bilateral lung lavage can be performed safely and effectively in a pediatric patient with pulmonary alveolar proteinosis by isolating individual lungs using a dual-lumen endotracheal tube. In this patient, therapeutic bilateral lung lavage was found to have a significant impact on lung function and mechanics after this procedure.

摘要

目的

肺泡蛋白沉积症是一种罕见的疾病,可导致因表面活性蛋白在肺泡中过度积聚而引起的危及生命的呼吸衰竭。清除这些分泌物的标准治疗方法是通过治疗性双侧肺灌洗。在医学文献中,对于治疗性双侧肺灌洗的操作程序以及评估该侵入性程序在肺泡蛋白沉积症患儿中的整体有效性的方法,尚无充分描述。我们成功且安全地对一名患有肺泡蛋白沉积症的儿童进行了治疗性双侧肺灌洗,并获得了肺力学测量值。

设计

病例报告。

设置

儿童医院手术室。

患者/受试者:一名 13 岁男孩,患有肺泡蛋白沉积症,因反复呼吸窘迫需要进行多次治疗性双侧肺灌洗。

干预措施

该患者因最近出现进行性呼吸困难、呼吸窘迫、肺功能测量值下降和影像学异常恶化而到医院手术室接受治疗性肺灌洗。我们在治疗性双侧肺灌洗前后获得了基础肺功能和呼吸系统顺应性测量值。第一天灌洗左肺,第三天灌洗右肺,使用选择性支气管内插管和选择性肺通气。

结果

该过程耐受良好,可清除大量积聚的分泌物。灌洗后,患者的呼吸窘迫、胸部 X 线表现、肺顺应性和肺功能测量值均有所改善。

结论

本病例报告表明,通过使用双腔气管内导管对单个肺进行隔离,治疗性双侧肺灌洗可安全有效地用于肺泡蛋白沉积症的儿科患者。在该患者中,该治疗性双侧肺灌洗在该程序后对肺功能和力学有显著影响。

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Whole lung lavage of nine children with pulmonary alveolar proteinosis: experience in a tertiary lung center.9例儿童肺泡蛋白沉积症的全肺灌洗:三级肺中心的经验
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