• Journal of Yeungnam Medical Science
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• v.27 no.1
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• pp.57-62
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• 2010

Pulmonary alveolar proteinosis (PAP) is a rare disorder that's characterized by accumulation of surfactant components in the alveolar space. Idiopathic PAP is recognized as an autoimmune disease that's due to impaired alveolar macrophage function and this caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). We report here a case of pulmonary alveolar proteinosis that was deemed interstitial lung disease at the initial diagnosis. A 61-year-old man presented with intermittent blood tinged sputum and dyspnea on exertion. The man was a painter for 30 years and he had a 10 pack-years smoking history. Chest computerized tomography (CT) revealed multifocal ground-glass opacity with interstitial thickening at both lungs. His pulmonary function tests and methacholine test revealed non specific results. He was diagnosed with interstitial lung disease on the basis of the chest CT finding and occupational history. However, seven months later, his symptoms progressed. Follow-up chest CT was performed. Wedge resection via video-assisted thoracoscopic surgery (the anterior basal segment of the left lower lobe) was done. Microscopic examination showed large groups of alveoli with excessive amounts of surfactant and a complex mixture of protein and lipid (fat) molecules. Finally, he was diagnosed as having pulmonary alveolar proteinosis.

• The Korean Journal of Cytopathology
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• v.11 no.2
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• pp.103-108
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• 2000

Pulmonary alveolar proteinosis(PAP) is a rare disease in which the alveolar spaces are filled with an eosinophilic, PAS-positive material, whereas the interstitial architecture of the lung usually remains unaffected. Although a definitive diagnosis is usually made by an open lung biopsy, bronchoalveolar lavage(BAL) cytology may play a decisive role in the diagnosis and therapy of these patients and may spare a patient a more invasive diagnostic procedure. The author presents a patient in whom BAL cytology specimen contained the characteristic globules of amorphous proteinaceous PAS-positive material accompanied by background of rare macrophages and inflammatory cells. Ultrastructural study using BAL specimen can confirm the diagnosis of PAP.

• Tuberculosis and Respiratory Diseases
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• v.56 no.6
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• pp.683-686
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• 2004

Pulmonary alveolar proteinosis is a rare disorder in which lipoproteinaceous material accumulates within the alveoli. We report a case of pulmonary alveolar proteinosis in a 41 year old female patient. She complained of a dry cough in the preceding 6 months. She presented symptoms of mild hypoxemia and diffuse infiltration at both lower lung fields. A milky fluid was obtained by bronchoalveolar lavage. We confirmed by light microscopic examinations of the lung tissues obtained by transbronchial lung biopsy. Through several follow-ups, the patients symptoms were mild.

• Journal of Yeungnam Medical Science
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• v.38 no.4
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• pp.374-380
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• 2021

Pulmonary alveolar proteinosis (PAP) is an uncommon disease characterized by progressive accumulation of lipoprotein material in the lungs due to impaired surfactant clearance. Whole-lung lavage (WLL) is the current standard treatment and consists of sequential lavage of each lung to mechanically remove the residual material from the alveoli. Although WLL is considered safe, unexpected complications can occur. Moreover, due to the rarity of the disease itself, this procedure is unknown to many physicians, and management of intraoperative complications can be challenging for anesthesiologists. Lung ultrasound (LUS) provides reliable and valuable information for detecting perioperative pulmonary complications and, in particular, quantitation of lung water content. There have been reports on monitoring the different stages of controlled deaeration of the non-ventilated lung during WLL using LUS. However, it has been limited to non-ventilated lungs. Therefore, we report the use of LUS in WLL to proactively detect pulmonary edema in the ventilated lung and implement a safe and effective anesthesia strategy. Given the limited diagnostic tools available to anesthesiologists in the operating room, LUS is a reliable, fast, and noninvasive method for identifying perioperative pulmonary complications in patients with PAP undergoing WLL.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.437-443
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• 1998

Pulmonary alveolar proteinosis is characterized by the accumulation of PAS positive lipoproteinaceous or amorphous proteinaceous material in the alveolar space with spared delicate septal architecture of the lung interstitium and impaired gas exchange of alveoli. We experienced a case of secondary pulmonary alveolar proteinosis in a 41 year old male patient who have occupational history of engagement as a mason over 4year. He compalined exertional dyspnea and chest discomfort, and presented fine inspiratory crackle at both lower lung field, numerous fine nodular denisties in both lung field with peripheral sparing. Light microscopic finding of lung tissue obtained by transbronchiallung biopsy revealed homogenous eosinophilic colloid-like luminal content in the alveolar space, and electron microscopy of bronchoalveolar lavage fluid concentrate showed electron-dense multilamellated structures. To treat the disease, we tried whole lung lavage of right lung with isotonic saline under general anesthesia. After whole lung lavage of right lung, he showed markid improvement of symptom and partial improvement of chest X-ray findings. The patient has been followed for 12 month until now, with no evidence of aggravation.

• Tuberculosis and Respiratory Diseases
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• v.63 no.3
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• pp.294-298
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• 2007

Pulmonary alveolar proteinosis is a rare disorder that is characterized by the abundant accumulation of surfactant-derived material within the alveolar spaces and distal bronchioles. Although the findings of homogenous ground-glass opacities overlaid by thickened septa, which form a "crazy paving" pattern on high-resolution computed tomography, can assist in making a diagnosis, a lung biopsy is usually required. This disorder has a variable clinical course, from spontaneous resolution to respiratory failure and death. While a whole lung lavage has been the standard treatment since the early 1960s, GM-CSF therapy has been attempted based on the recently suggested pathogenetic mechanism. We report a case of pulmonary alveolar proteinosis that resolved spontaneously after an open lung biopsy.

• Tuberculosis and Respiratory Diseases
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• v.67 no.6
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• pp.569-573
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• 2009

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Idiopathic PAP has recently been recognized as a autoimmune disease of impaired alveolar macrophage function caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). While whole lung lavage has been the standard treatment, not every patient shows a complete response. Subcutaneous injection or inhalation of GM-CSF is another promising treatment option for PAP. A 45-year-old patient visited our hospital for dyspnea, he was diagnosed as PAP and underwent whole lung lavage. Eighteen months later, the patient had not achieved complete remission in despite of initial response. After then he was administered with GM-CSF (5 ${mu}g/kg/day$, subcutaneous injection) for fivetimes a week during 2 months. Nine months later, the abnormal shadows in high-resolution computed tomography (HRCT) decreased and the patient fully recovered in forced vital capacity. After 60 months, the HRCT scan showed complete remission of PAP.

• Tuberculosis and Respiratory Diseases
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• v.52 no.4
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• pp.411-418
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• 2002

Pulmonary alveolar proteinosis(PAP) is a disorder in which an insoluble, proteinaceous material, rich in phospholipids, is deposited in the alveoli and bronchioles. The deficiency in the clearance and degradation of the intra-alveolar phospholipoproteinaceous material in PAP most likely represents a dysfunction of the type II pneumocytes. Although the pathogenesis and causative treatment of PAP is unclear a whole lung bronchopulmonary lavage is a relatively safe and effective treatment. Here we experienced a case of pulmonary alveolar proteinosis in a 62 year old female patient who had pulmonary tuberculosis approximately 20 years ago. She complained of aggravated dyspnea and chronic cough, and presented fine inspiratory crackles at both lung fields, diffuse ground glass opacity with some area of consolidation and smooth interlobular septal thickenings in both upper, right middled lobes, and a portion of right lower lobe. Optical microscopy of the lung tissue obtained by and open lung biopsy revealed many granulomas containing acid-fast smear positive bacilli and diffuse homogeneous PAS-positive fluid in the alveolar space. Immunohistochemical stain showed surfactant. A in the alveolar space. Antituberculosis drugs with bronchoalveolar lavage were used to treat the disease. Thereafter she showed improvement in her symptoms and a partial improvement in the chest X-ray and HRCT findings. We present a case of PAP associated with pulmonary tuberculosis.

• Journal of Yeungnam Medical Science
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• v.37 no.1
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• pp.67-72
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• 2020

Whole lung lavage (WLL) is a therapeutic procedure to remove accumulated material by infusing and draining the lungs with lavage fluid. This procedure has been regarded as the current standard of care to treat pulmonary alveolar proteinosis. However, the WLL protocol has not yet been standardized and the technique has been refined and modified a number of times. A rapid infusion system is a device used to infuse blood or other fluids at precise rates and normothermic conditions. This device is not typically used in WLL, which relies on the passive infusion of fluids using the gravitational force. However, in this study we performed WLL using a rapid infusion system, since we aimed to take advantage of its shorter operation time and greater degree of control over fluid volume and temperature. The patient's symptoms improved without the occurrence of any complications.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.860-862
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• 2005

We describe a case of pulmonary alveolar proteinosis in a male adult with lung cancer To achieve the successful operation of lung cancer, we used percutaneous veno-venous extracorporeal membrane oxygenation (ECMO) during whole lung lavage (WLL) of the contralateral lung. We performed successful WLL under ECMO support.