Pulmonary Alveolar Proteinosis Syndrome.

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 Med

authors

Suzuki T,Trapnell BC

doi

10.1016/j.ccm.2016.04.006

subject

Has Abstract

pub_date

2016-09-01 00:00:00

pages

431-40

issue

3

eissn

0272-5231

issn

1557-8216

pii

S0272-5231(16)30043-0

journal_volume

37

pub_type

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