title: 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.
authors: Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, MLC7029, 3333 Burnet Avenue, Cincinnati, OH 45229, USA. Electronic address: Takuji.Suzuki@cchmc.org.
authors: Takuji
authors: T
authors: Suzuki
authors: Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, MLC7029, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
authors: Bruce C
authors: BC
authors: Trapnell
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D018893:Bronchoalveolar Lavage; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology* / Q000627:therapeutic use; D006801:Humans; D016676:Macrophages, Alveolar / Q000276:immunology*; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism / Q000628:therapy; D011663:Pulmonary Surfactants / Q000378:metabolism*
chemical_list: D001323:Autoantibodies; D011663:Pulmonary Surfactants; D016178:Granulocyte-Macrophage Colony-Stimulating Factor
keywords: Alveolar macrophages; CSF2RA; CSF2RB; GM-CSF; GM-CSF autoantibody; GM-CSF inhalation therapy; Pulmonary surfactant; Whole lung lavage