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Pulmonary alveolar proteinosis

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https://www.readbyqxmd.com/read/28421817/sequential-gm-csf-inhalation-after-whole-lung-lavage-for-pulmonary-alveolar-proteinosis-a-report-of-five-intractable-cases
#1
Shinya Ohkouchi, Keiichi Akasaka, Toshio Ichiwata, Shu Hisata, Hideya Iijima, Toshinori Takada, Hiroki Tsukada, Hideaki Nakayama, Jun-Ichi Machiya, Toshiya Irokawa, Hiromasa Ogawa, Yoko Shibata, Masakazu Ichinose, Masahito Ebina, Toshihiro Nukiwa, Hajime Kurosawa, Koh Nakata, Ryushi Tazawa
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by the excessive accumulation of surfactant proteins within the alveolar spaces and by higher titers of autoantibodies to granulocyte/macrophage colony stimulating factor (GM-CSF) in the serum and bronchoalveolar lavage fluid (BALF). The antibodies inhibit the maturation and phagocytosis of alveolar macrophages (AMs). Although the standard therapy for aPAP has been whole-lung lavage (WLL), this procedure is invasive and needs to be repeated for several years...
April 19, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28393663/herpes-simplex-virus-cytomegalovirus-and-pneumocystis-jiroveci-pneumonia-in-a-treatment-naive-hiv-positive-patient-with-pulmonary-alveolar-proteinosis-case-report
#2
Anita Bapat, Eliahu Bishburg, Sandhya Nagarakanti
Infection with multiple pathogens concurrently has become less common since the introduction of potent antiretroviral agent and effective prophylactic agents. We describe a patient with pulmonary alveolar proteinosis (PAP) admitted with pneumonia who was found to have AIDS and diagnosed with Pneumocystis jiroveci pneumonia, human herpesvirus type 1 (HHV-1), and a concomitant cytomegalovirus viremia. Polymerase chain reaction viral load was used for diagnosis of HHV-1 and follow-up. The patient was treated with trimethoprim-sulfamethoxazole and ganciclovir and had a resolution of pneumonia...
January 1, 2017: Journal of the International Association of Providers of AIDS Care
https://www.readbyqxmd.com/read/28379131/-pulmonary-alveolar-proteinosis-role-of-gm-csf-antibodies
#3
REVIEW
Glenda Ernst, Alejandro Salvado, Pedro Grynblat, Gabriela Tabaj, Oscar Garcia, Victor Hugo Cambursano, P Young
Pulmonary alveolar proteinosis is a rare illness characterized by alterations in the normal depuration of surfactants from the alveolar space in a process dependent of the granulocyte-monocyte colony stimulating factor (GM-CSF). The most of the patients develop antibodies that neutralize this factor, avoiding the normal homeostasis of surfactants. Therapeutic options include the total lung washes and administration of GM-CSF when the washes fail. Despite the contribution related with the knowledge about of the nature of the disease, the measurement of serum antibodies anti-GM-CSF is not a routine technique...
2017: Revista de la Facultad de Ciencias Médicas
https://www.readbyqxmd.com/read/28301535/quantitative-assessment-of-pulmonary-alveolar-proteinosis-pap-with-ultra-dose-ct-and-correlation-with-pulmonary-function-tests-pfts
#4
Xin Sui, Qianni Du, Kai-Feng Xu, Xinlun Tian, Lan Song, Xiao Wang, Xiaoli Xu, Zixing Wang, Yuyan Wang, Jun Gu, Wei Song, Zhengyu Jin
BACKGROUND: The purpose of this study was to investigate whether ultra-low-dose chest computed tomography (CT) can be used for visual assessment of CT features in patients with pulmonary alveolar proteinosis (PAP) and to evaluate the relationship between the quantitative analysis of the ultra-low-dose CT scans and the pulmonary function tests (PFTs). METHODS: Thirty-eight patients (mean [SD] age, 44.47 [12.28] years; 29 males, 9 females) with PAP were enrolled and subjected to two scans each with low-dose CT (reference parameters: 120 kV and 50 mAs) and ultra-low-dose CT (reference parameters, 80 kV, 25 mAs)...
2017: PloS One
https://www.readbyqxmd.com/read/28289977/location-function-and-ontogeny-of-pulmonary-macrophages-during-the-steady-state
#5
REVIEW
Natalio Garbi, Bart N Lambrecht
The lung is continuously exposed to potentially hazardous environmental challenges in the form of inert material and microbes. Pulmonary macrophages are critical in maintaining a low inflammatory context in the lung to facilitate optimal gas exchange. During infection, however, they mediate the immediate response to invading microorganisms in coordination with epithelial cells and other tissue-resident immune cells including dendritic cells, innate lymphocytes and memory T cells, and pulmonary interstitial macrophages...
March 13, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28275476/serum-kl-6-in-pulmonary-alveolar-proteinosis-china-compared-historically-with-germany-and-japan
#6
Wen-Liang Guo, Zi-Qing Zhou, Lu Chen, Zhu-Quan Su, Chang-Hao Zhong, Yu Chen, Shi-Yue Li
BACKGROUND: KL-6 is a biomarker of the severity of pulmonary alveolar proteinosis (PAP). We noticed a significant difference in the mean serum KL-6 level between Japanese and Caucasian patients. To assess the clinical value of serum KL-6 in Chinese PAP patients, and to compare the differences in serum KL-6 levels in Chinese patients and patients of other ethnicities. METHODS: From 2014-2016, we prospectively examined 37 Chinese Han patients with PAP, measured their serum KL-6 levels, evaluated the correlation between initial KL-6 levels and clinical variables, and compared our results with studies from Japan and Germany (similar methods were used)...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28270188/pulmonary-alveolar-proteinosis-and-first-successful-whole-lung-lavage-in-sri-lanka-a-case-report
#7
Janith Galhenage, Buddhika Weerasinghe, Wadasinghe Dilesha, Roshana Constantine, Bandu Gunasena
BACKGROUND: Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis. CASE PRESENTATION: We describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months' duration...
March 8, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28264879/abcg1-regulates-pulmonary-surfactant-metabolism-in-mice-and-men
#8
Thomas Q de Aguiar Vallim, Elinor Lee, David J Merriott, Christopher N Goulbourne, Joan Cheng, Angela Cheng, Ayelet Gonen, Ryan M Allen, Elisa N D Palladino, David A Ford, Tisha Wang, Ángel Baldán, Elizabeth J Tarling
Idiopathic pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of surfactant. Surfactant synthesis and secretion are restricted to epithelial type 2 pneumocytes (T2 cells). Clearance of surfactant is dependent upon T2 cells and macrophages. ATP Binding Cassette Transporter G1 (ABCG1) is highly expressed in both T2 cells and macrophages. ABCG1-deficient mice accumulate surfactant, lamellar body-loaded T2 cells, lipid-loaded macrophages, B-1 lymphocytes and immunoglobulins, clearly demonstrating ABCG1 has a critical role in pulmonary homeostasis...
March 6, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28230086/classification-of-idiopathic-interstitial-pneumonias-using-anti-myxovirus-resistance-protein-1-autoantibody
#9
Yoshimasa Hamano, Hiroshi Kida, Shoichi Ihara, Akihiro Murakami, Masahiro Yanagawa, Ken Ueda, Osamu Honda, Lokesh P Tripathi, Toru Arai, Masaki Hirose, Toshimitsu Hamasaki, Yukihiro Yano, Tetsuya Kimura, Yasuhiro Kato, Hyota Takamatsu, Tomoyuki Otsuka, Toshiyuki Minami, Haruhiko Hirata, Koji Inoue, Izumi Nagatomo, Yoshito Takeda, Masahide Mori, Hiroyoshi Nishikawa, Kenji Mizuguchi, Takashi Kijima, Masanori Kitaichi, Noriyuki Tomiyama, Yoshikazu Inoue, Atsushi Kumanogoh
Chronic fibrosing idiopathic interstitial pneumonia (IIP) can be divided into two main types: idiopathic pulmonary fibrosis (IPF), a steroid-resistant and progressive disease with a median survival of 2-3 years, and idiopathic non-specific interstitial pneumonia (INSIP), a steroid-sensitive and non-progressive autoimmune disease. Although the clinical courses of these two diseases differ, they may be difficult to distinguish at diagnosis. We performed a comprehensive analysis of serum autoantibodies from patients definitively diagnosed with IPF, INSIP, autoimmune pulmonary alveolar proteinosis, and sarcoidosis...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28216546/bach2-controls-homeostasis-of-eosinophils-by-restricting-the-type-2-helper-function-of-t-cells
#10
Yuki Sato, Hiroki Kato, Risa Ebina-Shibuya, Ari Itoh-Nakadai, Ryuhei Okuyama, Kazuhiko Igarashi
Bach2 is a transcription factor which represses its target genes and plays important roles in the differentiation of B and T lymphoid cells. Bach2-deficient (KO) mice develop severe pulmonary alveolar proteinosis, which is associated with increased numbers of granulocytes and T cells. Bach2 is essential for the regulation of T cells, but its role in the regulation of granulocytes is not clear. Here, we observed increased numbers of eosinophils but not neutrophils in the bone marrow, spleen, peripheral blood, and bronchoalveolar lavage fluids of Bach2 KO mice compared with those of wild-type (WT) mice...
March 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28212655/elderly-onset-hereditary-pulmonary-alveolar-proteinosis-and-its-cytokine-profile
#11
Masayuki Ito, Kazuyuki Nakagome, Hiromitsu Ohta, Keiichi Akasaka, Yoshitaka Uchida, Atsushi Hashimoto, Ayako Shiono, Toshinori Takada, Makoto Nagata, Jun Tohyama, Koichi Hagiwara, Minoru Kanazawa, Koh Nakata, Ryushi Tazawa
BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation, and is caused by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling. Abnormalities in CSF2 receptor alpha (CSF2RA) were reported to cause pediatric hereditary PAP. We report here the first case of CSF2RA-mutated, elderly-onset hereditary (h) PAP. CASE PRESENTATION: The patient developed dyspnea on exertion, and was diagnosed with PAP at the age of 77 years, based on findings from chest computed tomography scan and bronchoalveolar lavage...
February 17, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28202867/gm-csf-autoantibody-positive-pulmonary-alveolar-proteinosis-with-simultaneous-myeloproliferative-neoplasm
#12
REVIEW
Naoto Imoto, Nakashima Harunori, Katsuya Furukawa, Naoyuki Tange, Atsushi Murase, Masaya Hayakawa, Masatoshi Ichihara, Yosuke Iwata, Hiroshi Kosugi
Pulmonary alveolar proteinosis (PAP) is classified as autoimmune, secondary, or genetic. We herein describe a 69-year-old man with autoimmune PAP, simultaneously diagnosed with myeloproliferative neoplasm (MPN). Two years after the diagnosis, the MPN progressed to acute myeloid leukemia, and the patient died from an alveolar hemorrhage during remission induction chemotherapy. Throughout the clinical course, no progression of PAP was observed, despite the progression to leukemia. There are few reports of autoimmune PAP with hematological malignancy, and this case demonstrated that an evaluation for GM-CSF autoantibodies is important for distinguishing the autoimmune and secondary forms of PAP, even if the patient has hematological malignancy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28144129/spotlight-on-mavrilimumab-for-the-treatment-of-rheumatoid-arthritis-evidence-to-date
#13
REVIEW
Chiara Crotti, Maria Gabriella Raimondo, Andrea Becciolini, Martina Biggioggero, Ennio Giulio Favalli
The introduction of biological therapies into clinical practice has dramatically modified the natural history of chronic inflammatory diseases, such as rheumatoid arthritis (RA). RA is a systemic autoimmune disease that causes articular damage and has a great negative impact on patients' quality of life. Despite the wide spectrum of available biological treatments, ~30% of RA patients are still unresponsive, resulting in high disability and increased morbidity and mortality. In the last few decades, the scientific knowledge on RA pathogenesis vastly improved, leading to the identification of new proinflammatory molecules as potential therapeutic targets...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28136187/particle-induced-pulmonary-alveolar-proteinosis-and-subsequent-inflammation-and-fibrosis
#14
Ernst M Bomhard
This review analyzes the published data on cases of pulmonary alveolar proteinosis (PAP) in workers inhaling crystalline aluminum, indium, silicon, and titanium particles and possible sequelae, that is, inflammation and fibrosis, and compares these findings with those from animal experiments. In inhalation studies in rodents using crystalline indium and gallium compounds, pronounced PAP followed by inflammation and fibrosis down to very low concentration ranges have been reported. Crystalline aluminum, silicon, and titanium compounds also induced comparable pulmonary changes in animals, though at higher exposure levels...
January 1, 2017: Toxicologic Pathology
https://www.readbyqxmd.com/read/28118623/efficacy-of-whole-lung-lavage-in-pulmonary-alveolar-proteinosis-a-multicenter-international-study-of-gelf
#15
Pierre Gay, Benoit Wallaert, Stefan Nowak, Jonas Yserbyt, Stavros Anevlavis, Christophe Hermant, Alban Lovis, Olivier Menard, Bernard Maitre, Thomas Vandemoortele, Hervé Dutau, Amandine Briault, Arnaud Bourdin, Jean-Michel Vergnon, Marios E Froudarakis
BACKGROUND: New therapies have emerged in the treatment of pulmonary alveolar proteinosis (PAP) and, therefore, there is a real need to evaluate the efficacy of whole-lung lavage (WLL) in this rare disease. OBJECTIVES: The aim of this study was to assess the efficacy of WLL in patients with PAP. METHODS: We included 33 patients from 12 centers, which are members of the French-Speaking Thoracic Endoscopy Group, for analysis. Data collection concerned patients and disease characteristics, pulmonary function tests (PFTs) and technical information on the procedure...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28095804/pulmonary-manifestations-in-niemann-pick-type-c-disease-with-mutations-in-npc2-gene-case-report-and-review-of-literature
#16
Jayesh Sheth, Jijo John Joseph, Krati Shah, Mamta Muranjan, Mehul Mistri, Frenny Sheth
BACKGROUND: Niemann-Pick disease type C (NPC) is an inherited metabolic disorder; due to defect in cellular cholesterol trafficking. It is clinically a heterogeneous disease with variable age of onset with multiple organ systems being involved. NPC1 gene is involved in 95% cases where as remaining ~5% cases are linked with NPC2 gene. CASE PRESENTATION: Case-1, a 14-months-old female presented with recurrent respiratory distress, failure to thrive and hepatosplenomegaly...
January 17, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28088396/pulmonary-alveolar-proteinosis-in-association-with-secondary-hemophagocytic-lymphohistiocytosis
#17
Jenny Lin, Aliva De, Lisa Figueiredo, Rochelle Maxwell, Emily Wasserman, Kelly Adams, Jacqueline Weingarten, Giles Peek, Michael Miksa
Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease in the pediatric population. There are currently few cases documenting hemophagocytic lymphohistiocytosis as a cause for secondary PAP. We describe an ex-preterm child with secondary hemophagocytic lymphohistiocytosis, complicated by PAP and hypoxemic respiratory failure.
January 12, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28057010/update-on-lysinuric-protein-intolerance-a-multi-faceted-disease-retrospective-cohort-analysis-from-birth-to-adulthood
#18
Wladimir Mauhin, Florence Habarou, Stéphanie Gobin, Aude Servais, Anaïs Brassier, Coraline Grisel, Célina Roda, Graziella Pinto, Despina Moshous, Fahd Ghalim, Pauline Krug, Nelly Deltour, Clément Pontoizeau, Sandrine Dubois, Murielle Assoun, Louise Galmiche, Jean-Paul Bonnefont, Chris Ottolenghi, Jacques de Blic, Jean-Baptiste Arnoux, Pascale de Lonlay
BACKGROUND: Lysinuric protein intolerance (LPI) is a rare metabolic disease resulting from recessive-inherited mutations in the SLC7A7 gene encoding the cationic amino-acids transporter subunit y(+)LAT1. The disease is characterised by protein-rich food intolerance with secondary urea cycle disorder, but symptoms are heterogeneous ranging from infiltrative lung disease, kidney failure to auto-immune complications. This retrospective study of all cases treated at Necker Hospital (Paris, France) since 1977 describes LPI in both children and adults in order to improve therapeutic management...
January 5, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28031836/pulmonary-alveolar-proteinosis-a-case-report-and-world-literature-review
#19
Armando J Huaringa, Wassem H Francis
Pulmonary alveolar proteinosis (PAP) is a lung disorder which was first described in 1958 by Rosen et al. and is indeed rare disease with a prevalence of 0.1 per 100,000 individuals. PAP is characterized by abnormal accumulation of pulmonary surfactant in the alveolar space, which impairs gas exchange leading to a severe hypoxemia. Pulmonary surfactant is an insoluble proteinaceous material that is rich in lipids and stains positive with periodic acid-Schiff (PAS). The most common type of PAP is the so-called autoimmune or idiopathic type...
November 2016: Respirology Case Reports
https://www.readbyqxmd.com/read/28013480/disseminated-cryptococcosis-due-to-anti-granulocyte-macrophage-colony-stimulating-factor-autoantibodies-in-the-absence-of-pulmonary-alveolar-proteinosis
#20
Chen-Yen Kuo, Shang-Yu Wang, Han-Po Shih, Kun-Hua Tu, Wen-Chi Huang, Jing-Ya Ding, Chia-Hao Lin, Chun-Fu Yeh, Mao-Wang Ho, Shi-Chuan Chang, Chi-Ying He, Hung-Kai Chen, Chen-Hsuan Ho, Chen-Hsiang Lee, Chih-Yu Chi, Cheng-Lung Ku
INTRODUCTION: Autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) can cause acquired pulmonary alveolar proteinosis (PAP). Cases of acquired PAP susceptible to typical respiratory pathogens and opportunistic infections have been reported. Anti-GM-CSF autoantibodies have been reported in a few patients with cryptococcal meningitis. This study evaluated the presence of neutralizing anti-GM-CSF autoantibodies in patients without known congenital or acquired immunodeficiency with severe pulmonary or extrapulmonary cryptococcal infection but without PAP...
February 2017: Journal of Clinical Immunology
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