Surfactant metabolism dysfunction, pulmonary, 5

Common Name(s)

Surfactant metabolism dysfunction, pulmonary, 5

Pulmonary surfactant metabolism dysfunction-5 (SMDP5) is an autosomal recessive lung disorder manifest clinically and pathologically as pulmonary alveolar proteinosis (PAP). PAP is a rare lung disease characterized by the ineffective clearance of surfactant by alveolar macrophages. This results in the accumulation of surfactant-derived lipoproteinaceous material in the alveoli and terminal bronchioles, causing respiratory failure (summary by {2:Greenhill and Kotton, 2009}). For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 ({265120}).
 

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Condition Specific Organizations

Following organizations serve the condition "Surfactant metabolism dysfunction, pulmonary, 5" for support, advocacy or research.

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Scientific Literature

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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