Pulmonary alveolar proteinosis (PAP) or pulmonary alveolar phospholipoproteinosis, first described on 1958 by Rosen et al., is characterized by overproduction of abnormal, nonfunctio...
Diagnosis often delayed due to nonspecific presentation, therefore, high clinical suspicion and occupational and exposure history should be obtained.
Most common reported symptoms: dys...
Management is based on disease progression, coexisting infections, and degree of physiologic impairment (signs of poor gas exchange).
In general, patient is classified as asymptomatic or symp...
Serial serum LDH level, BAL IgG anti–GM-CSF concentration, and chest X-ray can correlate with disease activity. KL-6 in serum could be used for disease progressio...
European Management Platform: www.childeu.net www.childeu.net ??[View Abstract on OvidMedline]
PAP Foundation: http://www.papfoundation.org/ http://www.papfoundation.org/ ??[View Abs...
PAP is a rare disease characterized by accumulation of PAS-positive surfactant-like material in the alveolar spaces leading to impaired gas exchange.
Nonspecific respiratory symptoms an...
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