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Alveolar proteinosis

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https://www.readbyqxmd.com/read/28916727/inflammatory-responses-induce-an-identity-crisis-of-alveolar-macrophages-leading-to-pulmonary-alveolar-proteinosis
#1
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 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...
September 15, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28914826/pulmonary-alveolar-proteinosis-a-case-report
#2
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
#3
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
#4
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
#5
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, we 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28569052/serum-ykl-40-is-a-reliable-biomarker-for-pulmonary-alveolar-proteinosis
#13
Francesco Bonella, Xiaoping Long, Xuan He, Shinichiro Ohshimo, Matthias Griese, Josune Guzman, Ulrich Costabel
BACKGROUND AND OBJECTIVE: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar filling. YKL-40, a chitinase-like protein produced by macrophages and epithelial cells, is increased in patients with interstitial lung diseases. We aimed to evaluate the role of YKL-40 as a biomarker for PAP. METHODS: A total of 34 patients with autoimmune PAP and 50 healthy controls were studied. YKL-40 was measured by ELISA in serum and bronchoalveolar lavage fluid (BALF)...
May 31, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28552777/comparison-of-the-toxicity-of-sintered-and-unsintered-indium-tin-oxide-particles-in-murine-macrophage-and-epidermal-cells
#14
COMPARATIVE STUDY
Nicole S Olgun, Anna M Morris, Tabatha Lynn Barber, Aleksandr B Stefaniak, Michael L Kashon, Diane Schwegler-Berry, Kristin J Cummings, Stephen S Leonard
Indium-tin oxide (ITO) is used to produce flat panel displays and several other technology products. Composed of 90% indium oxide (In2O3) and 10% tin oxide (SnO2) by weight, ITO is synthesized under conditions of high heat via a process known as sintering. Indium lung disease, a recently recognized occupational illness, is characterized by pulmonary alveolar proteinosis, fibrosis, and emphysema. Murine macrophage (RAW 264.7) and epidermal (JB6) cells stably transfected with AP-1 to study tumor promoting potential, were used to differentiate between the toxicological profiles of sintered ITO (SITO) and unsintered mixture (UITO)...
September 15, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28514019/does-dust-associated-pulmonary-alveolar-proteinosis-represent-an-autoimmune-disorder
#15
Bilge Uzmezoglu, Cebrail Simsek, Sevtap Gulgosteren, Berna E Gebesoglu
The role of autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in the development of secondary pulmonary alveolar proteinosis (PAP) in patients exposed to occupational and environmental dust remains unclear. Herein, we describe two cases of secondary PAP who had GM-CSF antibodies and absence of STAT5 phosphorylation index, suggestive of a potential relationship between the appearance of GM-CSF antibodies and environmental dust exposure. However, whether the presence of GM-CSF antibodies is a part of the pathological process or represents an epiphenomenon is currently unknown...
June 2017: American Journal of Industrial Medicine
https://www.readbyqxmd.com/read/28512724/congenital-pulmonary-alveolar-proteinosis-from-birth-to-ten-years-of-age
#16
Sandra Alavuk Kundović, Ljiljana Popović
Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange. The disease is classified into congenital, secondary, and acquired. The congenital form includes inborn errors of surfactant metabolism, lysinuric protein intolerance and mutations in the components of granulocyte-macrophage colony-stimulating factor receptor. The main symptoms are non-specific. The radiologic appearance of pulmonary alveolar proteinosis is bilateral, symmetric and perihilar airspace consolidation...
May 17, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28507378/case-series-of-rare-interstitial-lung-disease-ild
#17
Naveed Haroon Rashid, Saba Farooq, Mohammad Ahmed, Ali Bin Sarwar Zubairi
Pulmonary alveolar proteinosis (PAP) is a rare lung disease where periodic acid Schiff (PAS)-positive eosinophilic material accumulates in the alveoli of the lungs. Here we describe two cases of young males who presented with dynpnoea and weight loss. The HRCT scan of the chest in both cases showed the typical "crazy-paving" pattern and lung biopsies confirmed the diagnosis of PAP. They showed remarkable symptomatic improvement with therapeutic whole lung lavage.
May 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28500076/stat5-is-required-for-cd103-dendritic-cell-and-alveolar-macrophage-development-and-protection-from-lung-injury
#18
William E Eddy, Ke-Qin Gong, Bryan Bell, William C Parks, Steven F Ziegler, Anne M Manicone
We tested the role of Stat5 in dendritic cell and alveolar macrophage (AM) homeostasis in the lung using CD11c-cre mediated deletion (Cre(+)5(f/f)). We show that Stat5 is required for CD103(+) dendritic cell and AM development. We found that fetal monocyte maturation into AMs was impaired in Cre(+)5(f/f) mice, and we also confirmed impaired AM development of progenitor cells using mixed chimera experiments. In the absence of Stat5 signaling in AMs, mice developed alveolar proteinosis with altered lipid homeostasis...
June 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28489417/a-semiquantitative-ct-grading-system-for-evaluating-therapeutic-response-in-pulmonary-alveolar-proteinosis
#19
Sayoko Tokura, Masanori Akira, Tomohisa Okuma, Ryushi Tazawa, Toru Arai, Chikatoshi Sugimoto, Akiko Matsumuro, Masaki Hirose, Toshinori Takada, Koh Nakata, Haruyuki Ishii, Yasunori Kasahara, Masayuki Hojo, Shinya Ohkouchi, Yoshiko Tsuchihashi, Masanori Yokoba, Ryosuke Eda, Hideaki Nakayama, Takahito Nei, Konosuke Morimoto, Yasuyuki Nasuhara, Masahito Ebina, Toshio Ichiwata, Koichiro Tatsumi, Etsuro Yamaguchi, Yoshikazu Inoue
RATIONALE: A useful semi-quantitative method of using CT images to evaluate therapeutic response in pulmonary alveolar proteinosis (PAP) has not been established, although the extent score or grading score of ground-glass opacities has been used. OBJECTIVES: The purpose of this study was to establish a semi-quantitative method for evaluating therapeutic response in PAP. METHODS: CT scans obtained within one month before and after the therapy from 32 patients with PAP who participated in a multicenter phase 2 trial of GM-CSF inhalation therapy were evaluated by two chest radiologists independently...
May 10, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28489415/hepatic-steatosis-accompanies-pulmonary-alveolar-proteinosis
#20
Alan N Hunt, Anagha Malur, Tual Monfort, Pavlos Lagoudakis, Sumeet Mahajan, Anthony D Postle, Mary Jane Thomassen
Maintenance of tissue-specific organ lipid compositions characterises mammalian lipid homeostasis. Lung and liver synthesise mixed phosphatidylcholine (PC) molecular species subsequently "tailored" for function. Lungs progressively enrich disaturated PC (DSPC) directed to lamellar body (LB) surfactant stores prior to secretion. Liver accumulates polyunsaturated PC directed to VLDL assembly and secretion, or triglyceride stores. In each tissue, selective PC species enrichment mechanisms lie at the heart of effective homeostasis...
May 10, 2017: American Journal of Respiratory Cell and Molecular Biology
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