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Pulmonary alveolar proteinosis

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https://www.readbyqxmd.com/read/29162083/clinical-significance-of-cigarette-smoking-and-dust-exposure-in-pulmonary-alveolar-proteinosis-a-korean-national-survey
#1
Ji An Hwang, Joo Han Song, Jung Hoon Kim, Man Pyo Chung, Dong Soon Kim, Jin Woo Song, Young Whan Kim, Sun Mi Choi, Seung Ick Cha, Soo Taek Uh, Choon-Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Yangjin Jegal, Hyun Kyung Lee, Jong Sun Park, Moo Suk Park
BACKGROUND: This study aimed to investigate clinical characteristics of Korean PAP patients and to examine the potential risk factors of PAP. METHODS: We retrospectively reviewed medical records of 78 Korean PAP patients diagnosed between 1993 and 2014. Patients were classified into two groups according to the presence/absence of treatment (lavage). Clinical and laboratory features were compared between the two groups. RESULTS: Of the total 78 PAP patients, 60% were male and median age at diagnosis was 47...
November 21, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29135404/-bilateral-total-lung-lavage-in-a-patient-with-pulmonary-alveolar-proteinosis
#2
Müge Erbay, Yılmaz Bülbül, Funda Öztuna, Mehmet Kılıç, Neslihan Özçelik, Şafak Ersöz
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant composed of proteins and lipids. Three main categories of PAP have been defined depending on the aetiology: primer/idiopathic, neonatal/congenital, secondary and exogenous/environmental exposure. Radiologically diffuse ground glass opacities, interlobular and intralobular septal thickening is seen. Although open lung biopsy is accepted as the gold standard in diagnosis, it can be diagnosed by showing bronchoalveolar lavage (BAL) fluid with a milky appearance and periodic acid-schiff (PAS) positive globules in biopsy with clinical and radiological findings...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29123113/talen-mediated-functional-correction-of-human-ipsc-derived-macrophages-in-context-of-hereditary-pulmonary-alveolar-proteinosis
#3
Alexandra Kuhn, Mania Ackermann, Claudio Mussolino, Toni Cathomen, Nico Lachmann, Thomas Moritz
Hereditary pulmonary alveolar proteinosis (herPAP) constitutes a rare, life threatening lung disease characterized by the inability of alveolar macrophages to clear the alveolar airspaces from surfactant phospholipids. On a molecular level, the disorder is defined by a defect in the CSF2RA gene coding for the GM-CSF receptor alpha-chain (CD116). As therapeutic options are limited, we currently pursue a cell and gene therapy approach aiming for the intrapulmonary transplantation of gene-corrected macrophages derived from herPAP-specific induced pluripotent stem cells (herPAP-iPSC) employing transcriptional activator-like effector nucleases (TALENs)...
November 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29114417/an-alternative-lung-isolation-technique-in-paediatric-pulmonary-alveolar-proteinosis
#4
Tümay Umuroğlu, Merve Altıntaş, Tural Abdullayev, Gürsu Kıyan, Hilmi Ö Ayanoğlu
Lung isolation during the lung lavage of children with pulmonary alveolar proteinosis (PAP) poses challenges to anaesthesiologists. There is no established technique in the management of lung lavage in children; each described technique has its own advantages and disadvantages. We described a patient (2.5-year-old) with PAP, who has undergone left lung lavage. While his lung was isolated by a Fogarty catheter, lavage was performed via a feeding tube, and the right lung was ventilated with a rigid bronchoscope...
October 2017: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/29110133/recurrent-pulmonary-alveolar-proteinosis-in-an-immunocompromised-host
#5
Amrit K Kamboj, Patrick Hoversten, Thomas G Cotter, Subir Bhatia, Kannan Ramar
No abstract text is available yet for this article.
November 6, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/29095306/clinical-features-of-secondary-pulmonary-alveolar-proteinosis-associated-with-myelodysplastic-syndrome-two-case-reports
#6
Yin Liu, Lu Lu Chen, Yu Ying Qiu, Yong Long Xiao, Hou Rong Cai
RATIONALE: Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces. Secondary PAP can result from myelodysplastic syndrome (MDS). PATIENT CONCERNS: But most reports described a single case; here we reported 2 cases of PAP secondary to MDS. One case developed secondary PAP at the same time as MDS, and the other developed during the course of MDS. DIAGNOSES: The diagnosis of PAP was made by bronchoalveolar lavage and based on the identification of periodic acid-Schiff-positive proteinaceous material...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29021953/bilateral-lung-disease-extensive-and-diffuse-diagnosis-of-pulmonary-alveolar-proteinosis-by-bronchoscopic-cryobiopsy
#7
Sebastián Gando, Roberto Duré, Damián Violi, Bibiana Vazquez, Gonzalo Labarca, Sebastián Fernandez-Bussy
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the intra-alveolar accumulation of a proteinaceous phospholipid-laden material called surfactant. Clinically, this disease should be suspected with respiratory failure in association with a crazy paving pattern on high-resolution chest computed tomography. We report a 24-year-old gentleman who was referred to us for a history of respiratory failure, treatment with invasive ventilation and tracheostomy. His blood exams and biochemistry were normal...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28966213/elevated-serum-anti-gm-csf-antibodies-before-the-onset-of-autoimmune-pulmonary-alveolar-proteinosis-in-a-patient-with-sarcoidosis-and-systemic-sclerosis
#8
Mari Yamasue, Shin-Ichi Nureki, Yuko Usagawa, Tomoko Ono, Hiroyuki Matsumoto, Takamasa Kan, Jun-Ichi Kadota
Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea...
September 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28916727/inflammatory-responses-induce-an-identity-crisis-of-alveolar-macrophages-leading-to-pulmonary-alveolar-proteinosis
#9
Risa Ebina-Shibuya, Mitsuyo Matsumoto, Makoto Kuwahara, Kyoung-Jin Jang, Manabu Sugai, Yoshiaki Ito, Ryo Funayama, Keiko Nakayama, Yuki Sato, Naoto Ishii, Yasunobu Okamura, Kengo Kinoshita, Kohei Kometani, Tomohiro Kurosaki, Akihiko Muto, Masakazu Ichinose, Masakatsu Yamashita, Kazuhiko Igarashi
Pulmonary alveolar proteinosis (PAP) is a severe respiratory disease characterized by dyspnea caused by accumulation of surfactant protein. Dysfunction of alveolar macrophages (AMs), which regulate the homeostasis of surfactant protein, leads to the development of PAP; for example, in mice lacking BTB and CNC homology 2 (Bach2). However, how Bach2 helps prevent PAP is unknown, and the cell-specific effects of Bach2 are undefined. Using mice lacking Bach2 in specific cell types, we found that the PAP phenotype of Bach2-deficient mice is due to Bach2 deficiency in more than two types of immune cells...
November 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28914826/pulmonary-alveolar-proteinosis-a-case-report
#10
Luis Alberto Concepción-Urteaga, Luis Alejandro Rodríguez-Hidalgo, Jorge Luis Cornejo-Portella, Oscar Neri Alquizar-Horna, Daniel Anderson Aguilar-Villanueva, Marcio José Concepción-Zavaleta, Mario Gustavo Azañero-Luján
INTRODUCTION: Pulmonary alveolar proteinosis is a rare, diffuse interstitial lung disease, characterized by alveolar obstruction due to the accumulation of pulmonary surfactant. CLINICAL PRESENTATION: A 30-year-old male with progressively worsening dyspnea and non-productive cough for one year. He was a sugar cane plantation worker and had prior recurrent respiratory infections. Physical exam revealed cyanosis, and bilateral coarse and fine rales. Chest computed tomography showed diffuse crazy paving pattern...
September 14, 2017: Medwave
https://www.readbyqxmd.com/read/28861115/a-pediatric-case-of-relapsed-pulmonary-alveolar-proteinosis-despite-successful-whole-lung-lavage
#11
Seung Young Jin, Hye Ri Yun, Yun Jung Choi, Jun Dong Park, Jin Tae Kim, Chang Hyun Kang, Young Sik Park, Young Hun Choi, Woo Sun Kim, Dong In Suh
Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of PAP treated with WLL. An 11-year-old boy was admitted with the chief complaint of a dry cough lasting 6 months. He developed symptoms of dyspnea on exertion and had difficulty in climbing stairs. He was ultimately diagnosed with PAP through video-assisted thoracoscopic lung biopsy...
July 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28860566/targeting-cholesterol-homeostasis-in-lung-diseases
#12
Anthony Sallese, Takuji Suzuki, Cormac McCarthy, James Bridges, Alyssa Filuta, Paritha Arumugam, Kenjiro Shima, Yan Ma, Matthew Wessendarp, Diane Black, Claudia Chalk, Brenna Carey, Bruce C Trapnell
Macrophages are critical to organ structure and function in health and disease. To determine mechanisms by which granulocyte/macrophage-colony stimulating factor (GM-CSF) signaling normally maintains surfactant homeostasis and how its disruption causes pulmonary alveolar proteinosis (PAP), we evaluated lipid composition in alveolar macrophages and lung surfactant, macrophage-mediated surfactant clearance kinetics/dynamics, and cholesterol-targeted pharmacotherapy of PAP in vitro and in vivo. Without GM-CSF signaling, surfactant-exposed macrophages massively accumulated cholesterol ester-rich lipid-droplets and surfactant had an increased proportion of cholesterol...
August 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28854814/function-and-safety-of-lentivirus-mediated-gene-transfer-for-csf2ra-deficiency
#13
Miriam Hetzel, Takuji Suzuki, Anna Rafiei Hashtchin, Paritha Arumugam, Brenna Carey, Marc Schwabbauer, Alexandra Kuhn, Johann Meyer, Axel Schambach, Johannes Van Der Loo, Thomas Moritz, Bruce C Trapnell, Nico Lachmann
Hereditary pulmonary alveolar proteinosis (hPAP) is a rare disorder of pulmonary surfactant accumulation and hypoxemic respiratory failure caused by mutations in CSF2RA (encoding the granulocyte/macrophage colony-stimulating factor [GM-CSF] receptor α-chain [CD116]), which results in reduced GM-CSF-dependent pulmonary surfactant clearance by alveolar macrophages. While no pharmacologic therapy currently exists for hPAP, it was recently demonstrated that endotracheal instillation of wild-type or gene-corrected mononuclear phagocytes (pulmonary macrophage transplantation [PMT]) results in a significant and durable therapeutic efficacy in a validated murine model of hPAP...
August 30, 2017: Human Gene Therapy Methods
https://www.readbyqxmd.com/read/28794843/whole-lung-lavage-complicated-with-pneumothorax-a-case-report
#14
Hyun-Joo Ahn, Mikyung Yang, Jie Ae Kim, Burnyoung Heo, Jin-Kyoung Kim, So Yoon Park
A patient with pulmonary alveolar proteinosis underwent whole lung lavage of the right lung. Lavage of the left lung was not immediately possible because of severe hypoxemia. Three days later, after correction of hypoxemia, we re-attempted the left lung lavage. However, the patient had severe hypoxemia (SpO2 < 80%) within a few minutes of performing right one lung ventilation (OLV). On bronchoscopic examination, proper tube location was confirmed. Bronchodilator nebulization and steroid injection were attempted with no effect...
August 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/28771412/pulmonary-alveolar-proteinosis-a-comprehensive-clinical-perspective
#15
REVIEW
Matthias Griese
Pulmonary alveolar proteinosis is a broad group of rare diseases that are defined by the occupation of a lung's gas-exchange area by pulmonary surfactants that are not properly removed. The clinical and radiologic phenotypes among them are very similar. The age of manifestation plays a central role in the differential diagnosis of the almost 100 conditions and provides an efficient path to the correct diagnosis. The diagnostic approach is tailored to identify genetic or autoimmune causes, exposure to environmental agents, and associations with numerous other diseases...
August 2017: Pediatrics
https://www.readbyqxmd.com/read/28763266/insights-into-the-treatment-of-severe-pulmonary-alveolar-proteinosis
#16
Cliff Morgan
No abstract text is available yet for this article.
August 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28748093/pathological-features-of-explant-lungs-with-fibrosis-in-autoimmune-pulmonary-alveolar-proteinosis
#17
Manabu Ono, Ryoko Saito, Junya Tominaga, Yoshinori Okada, Shinya Ohkouchi, Tamiko Takemura
Association of fibrosis with autoimmune pulmonary alveolar proteinosis (aPAP) is rare. However, prognoses of such cases are poor and the process of the formation of fibrosis is still unknown. In this study, we report a case of aPAP with progressive fibrosis occurring in a 46-year-old woman. She had undergone several repetitions of whole lung lavage (WLL) for 7 years and granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation for 3 months; however, the progression of fibrosis was not hindered...
September 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28740686/whole-lung-lavage-technical-details-challenges-and-management-of-complications
#18
REVIEW
Ahmed Awab, Muhammad S Khan, Houssein A Youness
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with resulting hypoxemia and reduced lung function. Whole lung lavage (WLL) to physically remove the proteinaceous material from the affected lung is the standard treatment. Since its original description in 1964, there have been increasing numbers of WLL procedures done worldwide and the technique has been variously refined and modified. When done in experienced centers, WLL provides long lasting benefit in the majority of patients...
June 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28728273/-a-retrospective-study-of-the-perioperative-complications-following-whole-lung-lavage-in-the-treatment-of-pulmonary-alveolar-proteinosis
#19
Z Q Zhou, W L Guo, Y Chen, C H Zhong, S Y Li
Objective: To evaluate the safety of the procedures of whole lung lavage(WLL) for pulmonary alveolar proteinosis(PAP) in perioperative period. Methods: In this retrospective study, we collected clinical data from 78 WLL procedures of PAP patients from January 2006 to June 2016 in Guangzhou Institute of Respiratory Disease. The causes of perioperative complications were analyzed. Results: Eighteen (23.07%) of the 78 procedures developed complications, including pleural effusion(n=4), pneumonia(n=4), cardiac failure(n=2), cardiac arrhythmia (n=2), pneumothorax(n=2), atelectasis(n=1), lung edema(n=1), laryngeal edema(n=1), pleural effusion and pneumonia(n=1)...
July 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28595231/-pulmonary-alveolar-proteinosis-observed-in-the-subpleural-area-report-of-a-case
#20
Shin Shomura, Hitoshi Suzuki, Masaki Yada, Chiaki Kondo
A 39-year-old man consulted our hospital because of an abnormal shadow on a chest X-ray without any symptoms. A chest computed tomography revealed patchy peripheral ground-glass attenuation, in the subpleural area. Bronchoalveolar lavage fluid was clear and transbronchial lung biopsy findings were inconclutive. A video-assisted thoracic surgery-biopsy was performed. The specimens demonstrated accumulation of proteinaceous materials within alveolar spaces. The patient was given a diagnosis of pulmonary alveolar proteinosis...
June 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
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