Pulmonary alveolar proteinosis is a broad group of rare diseases that are defined by the occupation of a lung’s gas-exchange area by pulmonary surfactants that are not properly removed. The clinical and radiologic phenotypes among them are very similar. The age of manifestation plays a central role in the differential diagnosis of the almost 100 conditions and provides an efficient path to the correct diagnosis. The diagnostic approach is tailored to identify genetic or autoimmune causes, exposure to environmental agents, and associations with numerous other diseases. Whole-lung lavages are the cornerstone of treatment, and children in particular depend on the expertise to perform such therapeutic lavages. Other treatment options and long-term survival are related to the condition causing the proteinosis.
Pulmonary Alveolar Proteinosis: A Comprehensive Clinical Perspective
POTENTIAL CONFLICT OF INTEREST: The author has indicated he has no potential conflicts of interest to disclose.
FINANCIAL DISCLOSURE: The author has indicated he has no financial relationships relevant to this article to disclose.
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Matthias Griese; Pulmonary Alveolar Proteinosis: A Comprehensive Clinical Perspective. Pediatrics August 2017; 140 (2): e20170610. 10.1542/peds.2017-0610
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