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Pulmonary alveolar proteinosis

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https://www.readbyqxmd.com/read/28212655/elderly-onset-hereditary-pulmonary-alveolar-proteinosis-and-its-cytokine-profile
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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...
December 24, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27938549/-advance-in-pathogenesis-and-treatment-of-idiopathic-pulmonary-alveolar-proteinosis
#11
(no author information available yet)
No abstract text is available yet for this article.
December 12, 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/27890991/pulmonary-alveolar-proteinosis-experience-from-a-tertiary-care-center-and-systematic-review-of-indian-literature
#12
Vijay Hadda, Pawan Tiwari, Karan Madan, Anant Mohan, Nishkarsh Gupta, Sachidanand Jee Bharti, Vinod Kumar, Rakesh Garg, Anjan Trikha, Deepali Jain, Sudheer Arava, Gopi C Khilnani, Randeep Guleria
BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by deposition of lipoproteinaceous material within alveoli, with a variable clinical course. Here, we report an experience of management of PAP at our center. A systematic review of previously reported cases from India is also included in the article. MATERIALS AND METHODS: This study included patients with primary PAP managed at our center from 2009 to 2015. Diagnosis of primary PAP was based on histopathologic diagnosis on bronchoalveolar lavage or transbronchial lung biopsy and absence of causes of secondary PAP...
November 2016: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/27879496/death-due-to-pulmonary-alveolar-proteinosis
#13
Emran Altaf, Erik K Mitchel, Chris Berry, Altaf Hossain
Presented are 2 cases of death from pulmonary alveolar proteinosis (PAP). Within the past 2 years, there have been 2 cases of rare nonneoplastic lung disease that consists of the filling of the alveoli of the lung by a periodic acid-Schiff stain-positive lipoproteinaceous material. This condition bears a certain resemblance to interstitial lung disease and/or Pneumocystitis jirovecci infection of the lungs. The presented cases were clinically diagnosed as interstitial lung disease. In the first case presented, the decedent was admitted to hospital with diagnosis of pneumonia but died in hospital despite observation and treatment...
March 2017: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/27851574/1940-pulmonary-alveolar-proteinosis-requiring-ecmo-during-whole-lung-lavage
#14
Douglas Deever, Perry Nystrom
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27833966/analysis-of-bronchoalveolar-lavage-fluid-in-a-first-romanian-pulmonary-alveolar-proteinosis-cohort
#15
Oana Claudia Deleanu, Ana Maria Zaharie, Aneta Şerbescu, Floarea Mimi NiŢu, Florin Dumitru MihălŢan, Oana Cristina Arghir
The features of bronchoalveolar lavage fluid (BALF) components and the severity of pulmonary alveolar proteinosis (PAP) were analyzed in the first Romanian cohort of patients admitted to "Marius Nasta" Institute of Pneumophtisiology, Bucharest, Romania. A six-year follow-up study based on bronchoalveolar lavage (BAL) data was performed between January 2007 to December 2012. Study cohort consisted in 20 inpatients diagnosed with PAP, based on BALF cytological findings and÷or on histopathological findings. Demographic, medical history, tobacco use, clinical and radiological features, disease progression with or without whole lung lavage (WLL) therapy were collected...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27814844/pulmonary-alveolar-proteinosis-crazy-paving-to-whole-lung-lavage
#16
Shahnaz Ul Rehman, Jeffrey R Hammersley
No abstract text is available yet for this article.
November 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27783330/inhaled-sargramostim-induces-resolution-of-pulmonary-alveolar-proteinosis-in-lysinuric-protein-intolerance
#17
Laura M Tanner, Johanna Kurko, Maaria Tringham, Heikki Aho, Juha Mykkänen, Kirsti Näntö-Salonen, Harri Niinikoski, Heikki Lukkarinen
Pulmonary alveolar proteinosis (PAP) is a potentially fatal complication of lysinuric protein intolerance (LPI), an inherited disorder of cationic amino acid transport. The patients often present with mild respiratory symptoms, which may rapidly progress to acute respiratory failure responding poorly to conventional treatment with steroids and bronchoalveolar lavations (BALs). The pathogenesis of PAP in LPI is still largely unclear. In previous studies, we have shown disturbances in the function and activity of alveolar macrophages of these patients, suggesting that increasing the activity and the number of macrophages by recombinant human GM-CSF (rhuGM-CSF) might be beneficial in this patient group...
October 26, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27783217/lung-surfactant-metabolism-early-in-life-early-in-disease-and-target-in-cell-therapy
#18
REVIEW
Elena Lopez-Rodriguez, Gemma Gay-Jordi, Adele Mucci, Nico Lachmann, Anna Serrano-Mollar
Lung surfactant is a complex mixture of lipids and proteins lining the alveolar epithelium. At the air-liquid interface, surfactant lowers surface tension, avoiding alveolar collapse and reducing the work of breathing. The essential role of lung surfactant in breathing and therefore in life, is highlighted by surfactant deficiency in premature neonates, which causes neonatal respiratory distress syndrome and results in early death after birth. In addition, defects in surfactant metabolism alter lung homeostasis and lead to disease...
October 25, 2016: Cell and Tissue Research
https://www.readbyqxmd.com/read/27755185/anticytokine-autoantibodies-in-infection-and-inflammation-an-update
#19
Gabriela Barcenas-Morales, Peter Jandus, Rainer Döffinger
PURPOSE OF REVIEW: Concise overview of the field of anticytokine autoantibodies with a focus on recent developments. RECENT FINDINGS: Advances in particular in the analysis of autoantibodies to IFNγ, granulocyte-macrophage colony-stimulating factor (GM-CSF) and type I IFN are presented. The target epitope for anti-IFNγ autoantibodies has been found to have high homology to a protein from Aspergillus suggesting molecular mimicry as a mechanism of breaking self-tolerance...
December 2016: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27743827/-plasmapheresis-failure-in-the-treatment-of-auto-immune-pulmonary-alveolar-proteinosis
#20
A Jézéquel, M Kerjouan, M Lederlin, C Lainé-Caroff, C Camus, P Delaval, S Jouneau
INTRODUCTION: Auto-immune pulmonary alveolar proteinosis is a rare disorder characterized by the accumulation of surfactant proteins in the alveoli. CASE REPORT: We report a case of a 41-year-old smoker, presenting initially with acute respiratory failure. Whole lung lavages were effective initially but only for a few weeks. GM-CSF subcutaneous injections were not effective, and then plasmapheresis were tried. CONCLUSION: This is the fifth report of the use of this treatment in auto-immune pulmonary alveolar proteinosis...
October 13, 2016: Revue des Maladies Respiratoires
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