Pulmonary alveolar proteinosis

Ramirez, José; Harlan, William R.

doi:10.1016/0002-9343(68)90166-6

Cited by 109 publications

(23 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The fluid was re-aspirated through the catheter and evacuated by violent coughing [30]. Classical therapeutic bronchoalveolar lavage is performed under general anaesthesia in an operating room or an intensive care unit.…”

Section: Therapymentioning

confidence: 99%

Pulmonary alveolar proteinosis

Borie¹,

Danel²,

Mp³

et al. 2011

European Respiratory Review

239

258

View full text Add to dashboard Cite

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic ''crazy paving'' pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.

show abstract

Section: Therapymentioning

confidence: 99%

Pulmonary alveolar proteinosis

Borie¹,

Danel²,

Mp³

et al. 2011

European Respiratory Review

239

258

View full text Add to dashboard Cite

show abstract

“…Beginning with "segmental flooding" to physically remove the accumulated material, then extending the procedure to the first successful whole-lung lavage under local anesthesia, Ramirez et al revolutionized the concept of whole-lung lavage in pulmonary alveolar proteinosis. 151,152 Over the years this original procedure has been refined and modified to achieve good results with less procedural difficulty and fewer complications. 95,126,[153][154][155][156][157][158][159][160][161][162][163][164][165][166][167][168][169][170][171][172] The modifications include routine use of general anesthesia, 153,154 larger lavage volume, 154,155 lobar lavage, 162 positional clearance, 161 and chest percussion.…”

Section: Whole-lung Lavagementioning

confidence: 99%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

View full text Add to dashboard Cite

“…The aim is to eliminate the material in distal air spaces and restore the permeability of the alveolar-capillary barrier (14) . The use of whole-lung lavage is less well established in young infants and newborns mainly because of the technical difficulties associated with use of large endotracheal tube (15) . In coordination with a pediatric surgeon and a pediatric ENT surgeon we attempted alveolar lavage both for therapeutic and diagnostic purpose.…”

Section: Discussionmentioning

confidence: 99%