...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>BMC Pulmonary Medicine >Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report
【24h】

Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report

机译：远程治疗肺泡蛋白沉着症的联合疗法：一例报告

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Granulocyte macrophage colony-stimulating factor (GM-CSF) plays a role in the pathophysiology of PAP. We highlight a case of severe PAP treated with exogenous GM-CSF and sequential lobar lavage due to the unavailability of salvage methods of oxygenation. A 36?year old female was admitted with fevers, chills, and progressive dyspnea. On presentation she was tachypneic, tachycardic, and hypoxemic; labs revealed leukocytosis and lactic acidosis. Chest CT identified diffuse ground glass opacities in a ‘crazy-paving’ pattern. Following intubation due to impending respiratory failure, bronchoscopy with bronchoalveolar lavage was performed. The lavage return stained positive with Periodic Acid Schiff, confirming the diagnosis of PAP. Continued deterioration necessitated treatment; however, at this geographically remote center without ECMO services WLL was judged to carry significant risk. Nebulized GM-CSF was administered without significant improvement. Subcutaneous GM-CSF was administered and isolated subsegmental lavages of the bilateral upper lobes were performed, with rapid improvement in oxygenation. Additional sequential lobar lavage and continued GM-CSF therapy as an outpatient resulted in complete resolution of oxygen requirement and return to normal pulmonary physiology. The autoimmune form of PAP is the most common, indicating that therapy with GM-CSF may play an important role for many patients. Treatment with WLL may be impractical in some clinical settings due to the expertise and salvage modalities required. Sequential lobar lavage requires less specialized expertise and may incur less risk of refractory hypoxemia. We posit that this combined-modality therapy is ideally suited to geographically-remote centers such as our own.
机译：肺泡蛋白沉着症（PAP）是一种罕见的肺部疾病，其特征在于肺泡中磷脂蛋白物质的积累。表现是非特异性的，但通常包括呼吸困难;疾病范围包括快速进行性缺氧性呼吸衰竭。全肺灌洗（WLL）是有症状PAP的治疗选择，但短暂的氧合加重有时需要抢救方式，例如体外膜氧合（ECMO）。粒细胞巨噬细胞集落刺激因子（GM-CSF）在PAP的病理生理中起作用。由于缺氧的抢救方法不可用，我们重点介绍了使用外源性GM-CSF和连续大叶灌洗治疗的严重PAP病例。一名36岁的女性因发烧，发冷和进行性呼吸困难而入院。在介绍时，她表现为心动过速，心动过速和低氧血症。实验室发现白细胞增多和乳酸性酸中毒。胸部CT以“疯狂铺装”模式识别出弥漫性毛玻璃混浊。在由于即将发生的呼吸衰竭而进行插管后，进行了支气管肺泡灌洗术。灌洗液用高碘酸希夫染色呈阳性，证实了PAP的诊断。持续恶化需要治疗;但是，在这个没有ECMO服务的地理偏远中心，WLL被认为具有重大风险。雾化的GM-CSF给药没有明显改善。皮下注射GM-CSF并进行双侧上叶的分离节段灌洗，氧合迅速改善。在门诊期间进行额外的连续大叶灌洗并继续进行GM-CSF治疗可完全解决氧气需求并恢复正常的肺部生理功能。 PAP的自身免疫形式最为常见，这表明GM-CSF疗法可能对许多患者起重要作用。由于所需的专业知识和补救方式，在某些临床情况下使用WLL进行治疗可能不切实际。序贯的大叶灌洗需要较少的专业知识，并且可能导致难治性低氧血症的风险降低。我们认为，这种联合疗法非常适合于像我们这样的偏远地区中心。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号