• Journal of Yeungnam Medical Science
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• 제27권1호
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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.

• 대한세포병리학회지
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• 제11권2호
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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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• 제56권6호
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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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• 제38권4호
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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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• 제45권2호
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• pp.437-443
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• 1998

노작성 호흡곤란과 흉부 X-선 사진상의 이상소견으로 입원한 환자에서 직업력상의 규사폭로력과 조직검사에서의 폐포단백증의 소견으로 이차성 폐포단백증을 진단하고 전폐세척술의 시행으로 증상의 호전을 보인 1 예를 경험하였기에 문헌고찰과 함께 보고하였다.

• Tuberculosis and Respiratory Diseases
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• 제63권3호
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• pp.294-298
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• 2007

폐포단백증은 폐포에 표면활성제 기원의 인지질이 비정상적으로 다량 침착하는 드문 질병으로 자연 소실에서 호흡부전으로 인한 사망까지 다양한 임상경과를 보인다. 그 원인에 따라 선천성, 특발성, 이차성으로 나눌 수 있는데 GM-CSF의 작용경로의 이상이 중요한 병인으로 여겨지고 있다. 치료의 표준은 기관지 폐포세척술이며, 약 8%정도에서는 자연소실 되는 것으로 보고되었다. 저자들은 진단을 위해 흉강경하 쐐기 절제술을 시행하고 경과 관찰하던 중 자연 소실된 폐포단백증 환자를 경험하였기에 문헌고찰과 함께 이를 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제67권6호
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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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• 제52권4호
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• pp.411-418
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• 2002

저자 등은 폐결핵재발 후에 진행성의 호흡곤란과 만성기침을 호소하며 입원하여 흉부전산화 단층촬영 상에 폐포단백증의 특징적인 소견을 보이는 환자를 개흉 폐생검으로 확진하여 항결핵제 투여와 기관지폐포세척술로 임상적 호전을 보인 1예를 문헌고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제37권1호
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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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• 제38권12호
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• pp.860-862
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• 2005

폐포단백증과 폐암이 동반된 성인 남성의 경우에서 폐암의 절제를 위해서 술 전 폐세척이 필요하였다. 저자는 경피적 체외막 산소화를 이용한 폐세척으로 저산소증의 노출 없이 무사히 시행할 수 있었고 이어 폐암의 성공적인 절제를 경험하였기에 보고하는 바이다.