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Alveolar proteinosis

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
(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
#7
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
#8
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...
November 23, 2016: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/27851574/1940-pulmonary-alveolar-proteinosis-requiring-ecmo-during-whole-lung-lavage
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/27660154/transbronchial-cryobiopsy-in-diffuse-parenchymal-lung-disease-retrospective-analysis-of-74-cases
#16
Kamonpun Ussavarungsi, Ryan M Kern, Anja C Roden, Jay H Ryu, Eric S Edell
BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiologic, and pathologic features. Surgical lung biopsies remain the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic, Rochester, Minnesota in June 2013-September 2015...
September 19, 2016: Chest
https://www.readbyqxmd.com/read/27635117/a-new-scale-to-assess-the-severity-and-prognosis-of-pulmonary-alveolar-proteinosis
#17
JiuWu Bai, JinFu Xu, WenLan Yang, Beilan Gao, Weijun Cao, Shuo Liang, Huiping Li
Background. Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation. Small proportion of PAP patients experienced spontaneous remission. Objective. The aim of this study was to assess the severity and prognosis of PAP using various indexes. Methods. Characteristics, PaO2, lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale. Results. PaO2 was lower among smokers than among never-smokers...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/27595063/recurrence-of-pulmonary-alveolar-proteinosis-after-bilateral-lung-transplantation-in-a-patient-with-a-nonsense-mutation-in-csf2rb
#18
Masahiro Takaki, Takeshi Tanaka, Yoshihiro Komohara, Yoshiko Tsuchihashi, Daisuke Mori, Kentaro Hayashi, Junya Fukuoka, Naoya Yamasaki, Takeshi Nagayasu, Koya Ariyoshi, Konosuke Morimoto, Koh Nakata
Hereditary pulmonary alveolar proteinosis (PAP) caused by mutations in CSF2RA or CSF2RB, which encode GM-CSF receptor α and β respectively, is a rare disease. Although some experimental therapeutic strategies have been proposed, no clinical evidence has yet been reported. We herein describe the clinical course and recurrence of hereditary PAP after lung transplantation. A 36-year-old woman developed PAP of unknown etiology. She underwent bilateral lung transplantation from living donors at the age of 42 years because of severe respiratory failure complicated by pulmonary fibrosis...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27592713/successful-treatment-of-autoimmune-pulmonary-alveolar-proteinosis-in-a-pediatric-patient
#19
Mirjana Trukalj, Marija Perica, Željko Ferenčić, Damir Erceg, Marta Navratil, Gzim Redžepi, Boro Nogalo
BACKGROUND Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by the intra-alveolar accumulation of surfactant-derived material, which impairs gas exchange and results in respiratory insufficiency. Two major subtypes of PAP are autoimmune and non-autoimmune PAP. The diagnosis relies on clinical presentation, ground glass opacities on CT scan, bronchoscopy with PAS stain of BAL fluid (BALF), lung biopsy with PAS-positive material in the alveoli, and the presence of anti GM-CSF antibodies in serum or BALF for an autoimmune subtype...
September 5, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27586802/targeting-gm-csf-in-rheumatoid-arthritis
#20
REVIEW
Ali Berkant Avci, Eugen Feist, Gerd-Rüdiger Burmester
Granulocyte-macrophage colony-stimulating factor (GM-CSF) is well-known as a haemopoietic growth factor. However, it is also essential in regulating functions of mature myeloid cells such as macrophages. Preclinical studies and observations of flares of arthritis in patients following GM-CSF treatment supported its important contribution to the pathogenesis of rheumatoid arthritis (RA). As the most advanced compound, mavrilimumab, a monoclonal antibody against GM-CSF receptor, has already completed phase II trials with a long term of follow-up period of 74 weeks...
July 2016: Clinical and Experimental Rheumatology
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