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Clinical indications for bronchoscopy
Published in Don Hayes, Kara D. Meister, Pediatric Bronchoscopy for Clinicians, 2023
Katelyn Krivchenia, Meredith Merz Lind
The therapeutic utility of a flexible bronchoscope mostly revolves around its ability to suction, lavage, and instill medication in the more distal airways inaccessible to the rigid bronchoscope. The skilled clinician can clear various types and consistencies of secretions, mucus plugs, and airway casts (Figure 1.16) to improve ventilation and/or oxygenation. Serial lavages of normal saline can assist in clearing even more distal airways and can be utilized by experienced hands to treat pulmonary alveolar proteinosis.
Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
A 49 year old smoker goes to the GP with persistent cough. On examination he is noted to have clubbed fingernails. He has a plain film of the chest which reports marked symmetrical perihilar ground glass opacification. He is referred to the respiratory team who request a CT chest, which shows a crazy paving appearance. The team perform a bronchial lavage. The overall findings are highly suggestive for pulmonary alveolar proteinosis.
Lysinuric protein intolerance
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Pulmonary disease may be effectively treated with high-dose regimens of prednisolone [43], but some patients have not responded. Repeated whole-lung lavages are a successful approach for pulmonary alveolar proteinosis [61]. Heart-lung transplantation has not been recommended, because recurrence of disease in transplanted lungs has been reported [62]. Effective treatment of renal complications has not yet been reported.
Dusting off the numbers of in situ particle analysis: a 35-year experience
Published in Ultrastructural Pathology, 2019
John M. Carney, Elizabeth N. Pavlisko, Thomas A. Sporn, Victor L. Roggli
ISPA may also be useful in some less common differential diagnostic situations. Pulmonary alveolar proteinosis in a small percentage of cases is related to inhalation of excessive amounts of inorganic particulate material, usually silica but sometimes aluminum or indium as well.3,8 Identification of the implicated particulate material by ISPA may facilitate the diagnosis. Rare cases of diffuse interstitial fibrosis may be related to inhalation of rare earth dusts (cerium, lanthanum, neodymium, samarium). The diagnosis of rare earth pneumoconiosis would be difficult or impossible without the assistance of ISPA.9 In addition, we have previously reported a case of aluminum-induced pneumoconiosis (aluminosis) in a woman with rapidly progressive interstitial fibrosis (Figures 6–8).10 Some of these cases are quite rare because they represent a hypersensitivity or idiosyncratic reaction to the inhaled dusts.
Granulocyte Macrophage Colony-Stimulating Factor and whole lung lavage for pulmonary alveolar proteinosis: A synergistic effect?
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2022
Catherine Boily-Daoust, Marc Fortin, Alain Dumas, Jean Bussières
Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of pulmonary lipoproteinaceous material in the distal air spaces, resulting in progressive respiratory failure.1,2 Approximately 90% of adult PAP are autoimmune,3 with high level of autoantibodies against granulocyte macrophage colony-stimulating factor (GM-CSF). The classic treatment of PAP is whole lung lavage (WLL),4 in which large volumes of saline are instilled in each lung to remove the lipoproteinaceous material.5 Thirty to 50 percent of patients require only 1 lavage, while others need repeat lung lavages at intervals of 6 to 12 months.6,7
Mild dyspnea presenting as ‘crazy-paving’ on chest computed tomography
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
In our patient, autoimmune pulmonary alveolar proteinosis was suspected based on his initial CT findings and confirmed by subsequent pathologic and serologic testing. The presence of IgG anti-GM-CSF antibodies is highly sensitive and specific for autoimmune PAP. Given our patient’s mild symptoms, he was managed conservatively. Follow-up in 4 months revealed continuous mild shortness of breath with exertion still not meeting the criteria for whole lung lavage. Familiarity with the key clinical and diagnostic features of pulmonary alveolar proteinosis will help raise awareness and potential new treatment options.