Pulmonary alveolar proteinosis

Shattuck, Trisha M.; Bean, Sarah M.

doi:10.1002/dc.22857

Cited by 4 publications

(4 citation statements)

References 13 publications

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“…Aspirated foreign material and acellular structures like corpora amylacea need to be ruled out. 9 Electron microscopy provides irrefutable diagnosis of PAP with distinction from its differential diagnosis, but is not available in all set-ups. Takemura et al, 10 have grouped multilamellated structures in PAP into four types (A-D) based on their ultrastructural characteristics.…”

Section: Discussionmentioning

confidence: 99%

“…History of drug toxicity and cytological finding of vacuolated macrophages are quintessential for diagnosing drug related deposits. Aspirated foreign material and acellular structures like corpora amylacea need to be ruled out 9 . Electron microscopy provides irrefutable diagnosis of PAP with distinction from its differential diagnosis, but is not available in all set‐ups.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary alveolar proteinosis: Colourful crazy shapes and sizes on cytology

Gupta,

Kundu

2023

Diagnostic Cytopathology

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis: Colourful crazy shapes and sizes on cytology

Gupta,

Kundu

2023

Diagnostic Cytopathology

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“…Previously, an open biopsy was considered the criterion to establish the diagnosis of PAP. However, transbronchial biopsies or cytologic evaluation of bronchoalveolar lavage (BAL) samples are now routinely used to diagnose this disease [80]. …”

Section: Lipid Metabolism and Pulmonary Surfactantmentioning

confidence: 99%

Metabolic Functions of the Lung, Disorders and Associated Pathologies

Alvarado

Arce

2016

J Clin Med Res

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The primary function of the lungs is gas exchange. Approximately 400 million years ago, the Earth’s atmosphere gained enough oxygen in the gas phase for the animals that emerged from the sea to breathe air. The first lungs were merely primitive air sacs with a few vessels in the walls that served as accessory organs of gas exchange to supplement the gills. Eons later, as animals grew accustomed to a solely terrestrial life, the lungs became highly compartmentalized to provide the vast air-blood surface necessary for O2 uptake and CO2 elimination, and a respiratory control system was developed to regulate breathing in accordance with metabolic demands and other needs. With the evolution and phylogenetic development, lungs were taking a variety of other specialized functions to maintain homeostasis, which we will call the non-respiratory functions of the lung and that often, and by mistake, are believed to have little or no connection with the replacement gas. In this review, we focus on the metabolic functions of the lung, perhaps the least known, and mainly, in the lipid metabolism and blood-adult lung vascular endothelium interaction. When these functions are altered, respiratory disorders or diseases appear, which are discussed concisely, emphasizing how they impact the most important function of the lungs: external respiration.

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“…These pathologic findings were consistent with the clinical suspicion of PAP. 3,4 Further serologic testing demonstrated anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies, in keeping with autoimmune PAP. Her respiratory status significantly improved following the lavage.…”

mentioning

confidence: 96%

Bronchoalveolar Lavage Findings in a Pediatric Patient With Primary Pulmonary Alveolar Proteinosis

2019

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Pulmonary alveolar proteinosis (PAP) is an uncommon cause of respiratory failure characterized by abnormal accumulations of surfactant proteins within alveolar spaces. 1 PAP can be due to an autoimmune process or secondary to various systemic diseases including hematopoietic disorders, immune dysregulation, infections, inhalations, lysinuric protein intolerance, iatrogenic, or secondary to drugs. 2 Autoimmune PAP represents 90% to 95% of adult cases of PAP but is more rare in pediatrics, accounting for 5% to 10% of all cases. 2 In this article, we present a case of PAP in the pediatric setting, with serology confirming a primary autoimmune process. A 13-year-old female patient presented with a 6-month history of progressive dyspnea on exertion. On admission, she was found to be hypoxemic, requiring oxygen therapy. Chest computed tomography scan revealed diffuse groundglass opacities accompanied by inter-and intra-lobular septal thickening, a pattern classically described as "crazy paving." She proceeded to whole lung lavage requiring extracorporeal membrane oxygenation (ECMO) for both diagnostic and therapeutic indications. Microscopy demonstrated dense eosinophilic, amorphous globules (Figure 1A), which were periodic acid-Schiff-positive and diastase resistant (Figure 1B), in a background of acellular debris and scant inflammatory cells. Electron microscopy showed these globules to have concentric lamellations characteristic of surfactant bodies (Figure 1C). These pathologic findings were consistent with the clinical suspicion of PAP. 3,4 Further serologic testing demonstrated anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies, in keeping with autoimmune PAP. Her respiratory status significantly improved following the lavage. She was decannulated from ECMO and successfully extubated. She was discharged home with daily GM-CSF inhalations and nocturnal, supplemental oxygen with outpatient follow-up.

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Pulmonary alveolar proteinosis

Cited by 4 publications

References 13 publications

Pulmonary alveolar proteinosis: Colourful crazy shapes and sizes on cytology

Pulmonary alveolar proteinosis: Colourful crazy shapes and sizes on cytology

Metabolic Functions of the Lung, Disorders and Associated Pathologies

Bronchoalveolar Lavage Findings in a Pediatric Patient With Primary Pulmonary Alveolar Proteinosis

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