Abstract:
:Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by the accumulation of surfactant in alveoli and terminal airways resulting in respiratory failure. PAP comprises part of a spectrum of disorders of surfactant homeostasis (clearance and production). The surfactant production disorders are caused by mutations in genes required for normal surfactant production. The PAP syndrome is identified based on history, radiologic, and bronchoalveolar lavage and/or histopathologic findings. The diagnosis of PAP-causing diseases in secondary PAP requires further studies. Whole-lung lavage is the current standard therapy and promising new pharmacologic therapies are in development.
journal_name
Clin Chest Medjournal_title
Clinics in chest medicineauthors
Suzuki T,Trapnell BCdoi
10.1016/j.ccm.2016.04.006subject
Has Abstractpub_date
2016-09-01 00:00:00pages
431-40issue
3eissn
0272-5231issn
1557-8216pii
S0272-5231(16)30043-0journal_volume
37pub_type
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journal_title:Clinics in chest medicine
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