China
Africa
Explore chapters and articles related to this topic
Bronchoscopy for specific situations
Published in Don Hayes, Kara D. Meister, Pediatric Bronchoscopy for Clinicians, 2023
Douglas Sidell, Christopher T. Towe, MyMy C. Buu
Whole-lung lavage (WLL) or large-volume lung lavage is the primary treatment for certain forms of pulmonary alveolar proteinosis (PAP). It is also used to treat other alveolar-filling diseases such as lipoid pneumonia. PAP is a rare condition with accumulation of phospholipoproteinaceous material in the alveoli which impairs gas exchange, leading to respiratory insufficiency and hypoxic respiratory failure. Disturbance in surfactant homeostasis is caused by abnormal surfactant production, processing, and/or removal. Often, the lung is affected diffusely, with computed tomography (CT) of the chest showing ground glass opacities and interlobular and intralobular septal thickening. WLL attempts to clear this proteinaceous material, resulting in improvement of symptoms. Patients who are symptomatic and/or have impaired activities of daily life and/or have failure to thrive may be considered for WLL. The challenge of this intervention lies within the balance of providing respiratory support of the patient while administering lavage fluid to wash the affected lung at the same time. The approach is selective isolation of a single lung for washing and providing ventilation via the trachea to the contralateral lung. PAP can be primary or secondary. Patients may need repeated sessions of intervention to result in clinical improvement. The intervention may need to be repeated if the proteinaceous material re-accumulates.
Interstitial lung diseases
Published in Louis-Philippe Boulet, Applied Respiratory Pathophysiology, 2017
Dion Geneviève, Cormier Yvon, Boulet Louis-Philippe
Most patients will present nonspecific symptoms, mostly in the form of dyspnea and cough. Physical examination is often normal but cyanosis and clubbing are found in about a third of cases [159]. The chest radiograph shows bilateral symmetric alveolar opacities without air bronchogram. These images can mimic an acute pulmonary edema but there is no pleural effusion or cardiomegaly. The chest tomodensitometry reveals ground-glass opacification with thickened intra- and interlobular septa in typical polygonal shapes, referred to as the “crazy paving” pattern. BAL will yield typically a milky appearance liquid, rich in proteins and lipids and alveolar macrophages that are engorged with PAS-positive material. Whole lung lavage is the most efficient treatment [160], although new experimental therapies with GM-CSF have been used in patients with PALP with some success [161–164].
Predominantly Mature Interstitial Fibrosis
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
Thomas Sporn, Victor L. Roggli
Pulmonary alveolar proteinosis (PAP) is an alveolar filling process in which eosinophilic, granular, and periodic acid-Schiff (PAS)-positive proteinaceous material accumulates within the alveolar spaces. This disorder has both primary and secondary forms and may follow infection and immunosuppression, and is discussed further in chapter 8 . Treatment is with whole lung lavage that may be curative. Some patients, however, become refractory to lavage treatments and develop a more reticular fibrotic pattern on chest X ray in contrast to the predominantly airspace pattern early in the disease. The later stages are predominated by mature fibrosis and the histologic findings may be difficult to distinguish from UIP or fibrotic NSIP. Careful examination will usually disclose residual areas of alveolar filling by the characteristic PAS-positive material (Fig. 6), indicating the true underlying disease process resulting in late-stage fibrosis.
Power washing pulmonary alveolar proteinosis
Published in Baylor University Medical Center Proceedings, 2021
Tiana R. Endicott-Yazdani, Gary S. Schwartz, Haiying Zhang, Randall L. Rosenblatt, Puneet S. Garcha
Pulmonary alveolar proteinosis (PAP) is a rare source of dyspnea caused by alveolar lipoprotein accumulation.1,2 Imaging demonstrates interstitial thickening with ground glass infiltrates called “crazy paving” but is not pathognomonic. Alveoli fill with lipoprotein-rich material positive for periodic acid–Schiff on staining.3 Whole-lung lavage (WLL) is the standard of care; it ventilates one lung while the other is lavaged with large volume fluids to disimpact accumulated alveolar surfactant with the goal of improving oxygenation.4 Traditionally, lavage of each lung can be done separately. However, severely hypoxemic patients are precluded from lavaging even one lung.5 Veno-venous extracorporeal membrane oxygenation (VV-ECMO) has emerged as a means to provide oxygenation during WLL.6–8 We present our experience using VV-ECMO for WLL in three severely hypoxemic patients with PAP.
Pharmacotherapy options in pulmonary alveolar proteinosis
Published in Expert Opinion on Pharmacotherapy, 2020
Sabina Antonela Antoniu, Ruxandra Rajnoveanu, Mihaela Grigore, Ileana Antohe
Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease with an estimated prevalence of 7 cases per million inhabitants worldwide. It comprises a spectrum of etiologies ranging from genetic defects in the granulocyte-macrophage (GM-CSF) pathway (hereditary PAP, congenital PAP), autoimmune aggression caused by GM-CSF autoantibodies (aPAP and some forms of secondary PAP due to direct injury of alveolar macrophages (subsets of secondary PAP related to exposure to inhaled toxic compounds) [1–3]. The common denominator in all of these etiologies is abnormal pulmonary surfactant turnover associated with impaired alveolar macrophage function due to increased intracellular accumulation of phospholipids and cholesterol. Currently, the most commonly used therapeutic methods are whole-lung lavage and GM-CSF treatment. Lavage helps to clear the abnormal surfactant from the lungs but does not address the cause, and therefore must be done repeatedly. GM-CSF can be administered via subcutaneous injection or more recently via inhalation in patients with iPAP. However, in patients with severe forms of PAP this often only partially corrects the damage caused by GM-CSF autoantibodies. Alternative therapeutic options have been tested clinically or are in the experimental phase. This review will discuss these treatment options and offer opinions on their potential uses in specific circumstances.
Effect of a novel polyethylene glycol compound on lung lavage in dogs after the inhalation of depleted uranium dust
Published in International Journal of Radiation Biology, 2018
Jiong Ren, Yuhui Hao, Rui Gao, Ying Zhang, Yonghong Ran, Jing Liu, Xiaotian Dai, Wei Xiong, Yongping Su, Rong Li
The safety of whole-lung lavage (WLL) has been widely recognized in the treatment of lung diseases, such as pneumoconiosis, pulmonary alveolar proteinosis and fetal aspiration pneumonitis (Gay et al. 2017). WLL is a feasible treatment option for the inhalation of plutonium oxide (PuO2). Moreover, WLL can potentially be used to treat the inhalation of other insoluble radioactive materials. When the lung dose equivalent is relatively low, the risk of WLL to reduce stochastic effects should be carefully considered based on the experience of multiple cases. In Britain, Dean (Dean 1997) published a review to recommend the use of WLL when radionuclide intake exceeds the annual limit on intake (ALI). Gervelas et al. (2007) reported that diethylenetriamine-pentaacetic acid (DTPA) can be administered directly by aerosol inhalation into the lungs for lung and systemic decorporation of Pu. For rats contaminated by PuO2 inhalation, the application of a decorporation agent into the lungs increased the urine discharge of Pu 3-fold, without increasing the solubility of PuO2 in the lung. Thus far, few studies have reported the use of lung lavage and decorporation agents after the inhalation of DU dust, making it necessary to further investigate the decorporation of U dust inhaled into the lungs.