Pulmonary alveolar proteinosis (PAP) is a rare syndrome in which phospholipoproteinaceous matter accumulates in the alveoli leading to compromised gas exchange. Whole-lung lavage is considered the gold standard for severe autoimmune PAP and offers favorable long-term outcomes. In this case report, we describe the perioperative management and procedural specifics of a patient undergoing WLL for PAP in which an anesthesiologist serves as the proceduralist and a separate anesthesiologist provides anesthesia care for the patient.
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http://dx.doi.org/10.4103/aca.ACA_184_17 | DOI Listing |
Am J Respir Cell Mol Biol
January 2025
Duke Medicine, Medicine, Durham, North Carolina, United States.
Becoming more frequent due to climate change, ozone (O) exposures can cause lung injury. Alveolar type 2 (AT2) cells and hyaluronan (HA), a matrix component, are critical to repairing lung injury and restoring homeostasis. Here, we define the impact of HA on AT2 cells following acute O exposure.
View Article and Find Full Text PDFJ Thorac Dis
November 2024
Department of Respiratory Medicine, Alfred Hospital, Melbourne, Australia.
Background: The incidence of silicosis has increased due to occupational silica exposure from artificial stone, with no treatments proven to halt or reverse the disease. Whole lung lavage (WLL) involves the instillation of fluid into the lungs to wash out silica particles and disease-causing inflammatory cells. This study aimed to determine the feasibility, safety, and possible benefit of WLL in patients with artificial stone silicosis.
View Article and Find Full Text PDFERJ Open Res
November 2024
Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France.
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.
Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium.
J Clin Immunol
December 2024
Department of Blood and Marrow Transplantation, Bai Jerbai Wadia Hospital for Children, Mumbai, India, 400012.
Hereditary pulmonary alveolar proteinosis (hPAP) is a rare lung-related primary immunodeficiency. In hPAP, variants of genes encoding the heterodimeric GM-CSF receptor alpha or beta-chains (CSF2Rα, CSF2Rβ) lead to perturbations in GM-CSF signalling. These perturbations impair the scavenging function of pulmonary alveolar macrophages leading to accumulation of surfactant proteins and lipids within the alveoli.
View Article and Find Full Text PDFAANA J
December 2024
Clinical Assistant Professor, Department of Nurse Anesthesia Education, University of Kansas Medical Center, Kansas City, Kansas.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by the accumulation of surfactant/lipoprotein material in the alveoli and subsequent hypoxemic respiratory failure. Whole lung lavage (WLL), a procedure used to physically remove the lipoprotein material from the alveoli, is the first-line treatment for this disease process. Anesthesia providers may infrequently encounter the management of the WLL procedure due to the rarity of the underlying disease process.
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