Abstract:
BACKGROUND: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. CASE PRESENTATION: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. CONCLUSIONS: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.
journal_name
BMC Pulm Medjournal_title
BMC pulmonary medicineauthors
Boyce DSK,Lee JW,Shah P,Freeman JH,Aboudara MC,Hostler DCdoi
10.1186/s12890-019-0822-xsubject
Has Abstractpub_date
2019-03-12 00:00:00pages
61issue
1issn
1471-2466pii
10.1186/s12890-019-0822-xjournal_volume
19pub_type
杂志文章abstract:BACKGROUND:To investigate the use of do-not-resuscitate (DNR) orders in patients hospitalized with community-acquired pneumonia (CAP) and the association with mortality. METHODS:We assembled a cohort of 1317 adults hospitalized with radiographically confirmed CAP in three Danish hospitals. Patients were grouped into n...
journal_title:BMC pulmonary medicine
pub_type: 杂志文章
doi:10.1186/s12890-020-01236-1
更新日期:2020-07-24 00:00:00
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pub_type: 杂志文章
doi:10.1186/s12890-019-0938-z
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doi:10.1186/s12890-019-0866-y
更新日期:2019-06-17 00:00:00
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pub_type: 杂志文章
doi:10.1186/s12890-019-0926-3
更新日期:2019-08-27 00:00:00
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pub_type: 杂志文章
doi:10.1186/1471-2466-13-52
更新日期:2013-08-15 00:00:00
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journal_title:BMC pulmonary medicine
pub_type: 杂志文章,多中心研究
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pub_type: 杂志文章
doi:10.1186/s12890-018-0741-2
更新日期:2018-11-22 00:00:00
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pub_type: 杂志文章
doi:10.1186/1471-2466-10-52
更新日期:2010-10-20 00:00:00
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pub_type: 杂志文章,多中心研究
doi:10.1186/s12890-016-0331-0
更新日期:2016-12-01 00:00:00
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pub_type: 杂志文章
doi:10.1186/s12890-015-0004-4
更新日期:2015-02-22 00:00:00
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pub_type: 杂志文章
doi:10.1186/s12890-020-1089-y
更新日期:2020-03-06 00:00:00
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pub_type: 杂志文章
doi:10.1186/1471-2466-14-66
更新日期:2014-04-22 00:00:00
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pub_type: 杂志文章,多中心研究
doi:10.1186/s12890-017-0417-3
更新日期:2017-04-26 00:00:00
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pub_type: 杂志文章
doi:10.1186/s12890-018-0771-9
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doi:10.1186/s12890-020-01356-8
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pub_type: 杂志文章,meta分析,评审
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pub_type: 临床试验,杂志文章
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pub_type: 临床试验,杂志文章
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更新日期:2014-11-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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更新日期:2014-08-04 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2017-12-28 00:00:00
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pub_type: 杂志文章
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更新日期:2018-08-13 00:00:00
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pub_type: 杂志文章
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