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Experimental Model to Evaluate Resolution of Pneumonia
Published on: February 17, 2023
Pulmonary Alveolar Proteinosis: A Comprehensive Clinical Perspective
1Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University of Munich and the German Center for Lung Research, Munich, Germany matthias.griese@med.uni-muenchen.de.
Abstract:
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.
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