2,176 results match your criteria Alveolar Proteinosis


Inhaled granulocyte-macrophage colony stimulating factor for mild-to-moderate autoimmune pulmonary alveolar proteinosis - a six month phase II randomized study with 24 months of follow-up.

Orphanet J Rare Dis 2020 Jul 2;15(1):174. Epub 2020 Jul 2.

Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Background: Treatment of autoimmune pulmonary alveolar proteinosis (aPAP) by inhaled granulocyte-macrophage colony stimulating factor (GM-CSF) is considered safe and effective. Evidence of benefit from GM-CSG inhalation for mild to moderate aPAP patients is limited.

Methods: In this multicenter, randomized, open-labeled clinical trial, 36 aPAP patients with mild to moderate disease severity were randomized into either the GM-CSF treatment group or control group. Read More

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http://dx.doi.org/10.1186/s13023-020-01450-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7330972PMC

Krebs von den Lungen-6 as biomarker for disease severity assessment in interstitial lung disease: a comprehensive review.

Biomark Med 2020 Jul 2. Epub 2020 Jul 2.

Respiratory Diseases & Lung Transplantation, Department of Medical & Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy.

Interstitial lung diseases (ILD) are a group of lung disorders characterized by interstitial lung thickening. Krebs von den Lungen-6 (KL-6) is a molecule that is predominantly expressed by damaged alveolar type II cells and it has been proposed as a potential biomarker of different ILD. A growing literature about KL-6 has been reviewed and selected to evaluate its role in the clinical management of ILD to predict disease diagnosis, activity, prognosis and treatment response. Read More

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http://dx.doi.org/10.2217/bmm-2019-0545DOI Listing

Autoimmune Pulmonary Alveolar Proteinosis Complicated with Sarcoidosis: The Clinical Course and Serum Levels of Anti-granulocyte-macrophage colony-stimulating Factor Autoantibody.

Intern Med 2020 Jun 30. Epub 2020 Jun 30.

Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan.

Autoimmune pulmonary alveolar proteinosis (APAP) is caused by macrophage dysfunction due to anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibody. We experienced 2 cases of APAP complicated with sarcoidosis in a 42-year-old woman and a 51-year-old man (age at the sarcoidosis diagnosis). APAP preceded sarcoidosis in the woman, and both diseases were diagnosed simultaneously in the man. Read More

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http://dx.doi.org/10.2169/internalmedicine.3853-19DOI Listing

Indium oxide nanoparticles induce lung intercellular toxicity between bronchial epithelial cells and macrophages.

J Appl Toxicol 2020 Jul 1. Epub 2020 Jul 1.

Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, China.

Concerns have been raised over the safety and health of industrial workers exposed to indium oxide nanoparticles (IO-NPs) when working. IO-NPs were previously shown in vitro and in vivo to be cytotoxic, but the mechanism of pathogenesis was unclear. In this study, the effects of IO-NPs on lung cells associated with respiratory and immune barriers and the toxic effects of intercellular cascades were studied. Read More

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http://dx.doi.org/10.1002/jat.4023DOI Listing

Pulmonary Alveolar Proteinosis Due to in Type 1 Hyper-IgM Syndrome: A Case Report.

Front Pediatr 2020 11;8:264. Epub 2020 Jun 11.

Department of Pneumology, Zhejiang University School of Medicine of Children's Hospital, Hangzhou, China.

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease. Reports of rare cases of PAP due to () exist in infants with immunodeficiency diseases, but no cases have been reported to date in pediatric patients with type 1 hyper-IgM syndrome (HIGM1). Herein, we present a case of PAP secondary to on an infant with HIGM1. Read More

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http://dx.doi.org/10.3389/fped.2020.00264DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7301693PMC

The toxicology of gallium oxide in comparison with gallium arsenide and indium oxide.

Authors:
Ernst M Bomhard

Environ Toxicol Pharmacol 2020 Jun 19;80:103437. Epub 2020 Jun 19.

REACh ChemConsult GmbH, Strehlener Str. 14, D-01069 Dresden, Germany. Electronic address:

Gallium arsenide (GaAs) and indium oxide (InO) are used in electronic industries at high and increasing tonnages since decades. Gallium oxide (GaO) is an emerging wide-bandgap transparent conductive oxide with as yet little industrial use. Since GaAs has received critical attention due to the arsenic ion, it seemed reasonable to compare its toxicology with the respective endpoints of GaO and InO toxicology in order to find out if and to what extent arsenic contributes. Read More

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http://dx.doi.org/10.1016/j.etap.2020.103437DOI Listing

Interstitial lung diseases in children.

Presse Med 2020 Jun 18;49(2):103909. Epub 2020 Jun 18.

Pediatric pulmonology department, Trousseau hospital, reference center for rare lung diseases RespiRare, Assistance publique-Hôpitaux de Paris (AP-HP), , 75012 Paris, France; Sorbonne université and Inserm UMRS933, 75012 Paris, France. Electronic address:

Interstitial lung disease (ILD) in children (chILD) is a heterogeneous group of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. The pathogenesis of the various chILD is complex and the diseases share common features of inflammatory and fibrotic changes of the lung parenchyma that impair gas exchanges. The etiologies of chILD are numerous. Read More

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http://dx.doi.org/10.1016/j.lpm.2019.06.007DOI Listing

Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report.

Respir Med Case Rep 2020 30;30:101108. Epub 2020 May 30.

Division of Pulmonology, Geneva University Hospitals, 1211, Geneva, Switzerland.

Background: Pulmonary alveolar proteinosis (PAP) is a pulmonary disease characterized by disruption of surfactant homeostasis resulting in its accumulation in the alveoli. PAP is classically classified into three categories (Table 1): 1/primary (or autoimmune) with antibodies targeting the GM-CSF pathway, 2/secondary to another disease, typically a hematologic malignancy, and 3/genetic.

Case-report: A 30 year-old woman received an allogenic hematopoietic stem cell transplantation (HSCT) after treatment for acute myeloid leukemia (AML). Read More

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http://dx.doi.org/10.1016/j.rmcr.2020.101108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7276430PMC

Autoimmune pulmonary alveolar proteinosis mimicking pneumonia in an adolescent.

Respir Med Case Rep 2020 1;30:101100. Epub 2020 Jun 1.

Beaumont Health, 3601 W 13 Mile Road, Royal Oak, MI, 48073, USA.

Pulmonary alveolar proteinosis (PAP) is a rare disease of abnormal surfactant production and accumulation. It is typically divided into three main categories: autoimmune, secondary and genetic. The genetic type is more common in children and adolescents, while the autoimmune type is most commonly seen in adults. Read More

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http://dx.doi.org/10.1016/j.rmcr.2020.101100DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7270595PMC

Pharmacotherapy options in pulmonary alveolar proteinosis.

Expert Opin Pharmacother 2020 Jun 8:1-8. Epub 2020 Jun 8.

Faculty of Medicine, University of Medicine and Pharmacy Grigore T Popa , Iasi, Romania.

Introduction: Pulmonary alveolar proteinosis (PAP) is a heterogeneous group of rare diseases characterized by the abnormal production and impaired degradation of pulmonary surfactant as a result of malfunctioning of alveolar macrophages. This is due to the downstream dysregulation of the GM-CSF pathway, which can be caused by specific autoantibodies (autoimmune, aPAP formerly known as idiopathic iPAP), direct injury to alveolar macrophages (e.g. Read More

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http://dx.doi.org/10.1080/14656566.2020.1757650DOI Listing

A Global Slc7a7 Knockout Mouse Model Demonstrates Characteristic Phenotypes of Human Lysinuric Protein Intolerance.

Hum Mol Genet 2020 Jun 5. Epub 2020 Jun 5.

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA.

Lysinuric protein intolerance (LPI) is an inborn error of cationic amino acid (arginine, lysine, ornithine) transport caused by biallelic pathogenic variants in SLC7A7, which encodes the light subunit of the y+LAT1 transporter. Treatments for the complications of LPI, including growth failure, renal disease, pulmonary alveolar proteinosis, autoimmune disorders and osteoporosis, are limited. Given the early lethality of the only published global Slc7a7 knockout mouse model, a viable animal model to investigate global SLC7A7 deficiency is needed. Read More

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http://dx.doi.org/10.1093/hmg/ddaa107DOI Listing
June 2020
6.393 Impact Factor

[Therapeutic bilateral lung lavage in an adolescent with pulmonary alveolar proteinosis. Case report].

Arch Argent Pediatr 2020 06;118(3):e324-e328

Sección de Cuidados Respiratorios Pediátricos, Servicio de Kinesiología, Hospital Italiano de Buenos Aires, Ciudad de Buenos Aires, Argentina.

Pulmonary alveolar proteinosis is an unusual diffuse lung disease characterized by abnormal accumulation of pulmonary surfactant and lipoproteins in the alveolar space, which impairs gas exchange with a variable clinical course, ranging from an asymptomatic clinical presentation to severely affected respiratory failure. A 16-year-old girl with diagnosis of pulmonary alveolar proteinosis presented to our hospital for therapeutic lung lavage after a recent history of progressive dyspnea, respiratory distress, declining lung function measurements, and worsening radiographic abnormalities. We obtained baseline pulmonary function tests and laboratory measurements before and after therapeutic bilateral lung lavage. Read More

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http://dx.doi.org/10.5546/aap.2020.e324DOI Listing

Secondary Pulmonary Alveolar Proteinosis Associated with Primary Myelofibrosis and Ruxolitinib Treatment: An Autopsy Case.

Intern Med 2020 May 23. Epub 2020 May 23.

Department of Hematology, Oncology and Respiratory Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.

Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. Read More

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http://dx.doi.org/10.2169/internalmedicine.4082-19DOI Listing

Utilization of LC-MS to Determine Monoclonal Gammopathy-Associated Granulocyte Macrophage Colony Stimulating Factor Antibody and Novel Treatment of Pulmonary Alveolar Proteinosis.

J Appl Lab Med 2020 Mar;5(2):394-400

Department Internal Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN.

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http://dx.doi.org/10.1093/jalm/jfz024DOI Listing

Structure and activity of human surfactant protein D from different natural sources.

Am J Physiol Lung Cell Mol Physiol 2020 Jul 20;319(1):L148-L158. Epub 2020 May 20.

Department of Biochemistry, Faculty of Biology, Complutense University, Madrid, Spain.

Surfactant protein D (SP-D) is a C-type lectin that participates in the innate immune defense of lungs. It binds pathogens through its carbohydrate recognition domain in a calcium-dependent manner. Human surfactant protein D (hSP-D) has been routinely obtained from bronchoalveolar lavage of patients suffering from pulmonary alveolar proteinosis (PAP) and from amniotic fluid (AF). Read More

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http://dx.doi.org/10.1152/ajplung.00007.2020DOI Listing

Pioglitazone in pulmonary alveolar proteinosis: promising first clinical experience.

Respir Med Res 2020 Apr 18;78:100756. Epub 2020 Apr 18.

Assistance publique-Hôpitaux de Paris, Hôpital Bichat, Service de Pneumologie et Centre de Référence constitutif des Maladies Pulmonaires Rares, Département Hospitalo-Universitaire FIRE; Université Paris Diderot, Inserm UMR 1152, LabEx Inflamex, 75018 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.resmer.2020.100756DOI Listing

Primary plasma cell leukemia presenting as secondary pulmonary alveolar proteinosis.

Leuk Lymphoma 2020 May 13:1-4. Epub 2020 May 13.

Department of Clinical Therapeutics, School of Medicine, Alexandra General Hospital, National and Kapodistrian University of Athens, Athens, Greece.

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http://dx.doi.org/10.1080/10428194.2020.1761969DOI Listing

A Unique Case of Secondary Pulmonary Alveolar Proteinosis Following E-Cigarette, or Vaping, Product Use-Associated Lung Injury (EVALI).

Am J Respir Crit Care Med 2020 May 11. Epub 2020 May 11.

University of Rochester Medical Center, 6923, Pathology, Rochester, New York, United States;

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http://dx.doi.org/10.1164/rccm.202002-0252LEDOI Listing

Subcutaneous Sweet syndrome mimicking cellulitis in a patient with myelodysplastic syndrome and subsequent secondary pulmonary alveolar proteinosis.

Clin Exp Dermatol 2020 May 8. Epub 2020 May 8.

Departments of, Department of, Dermatology, Fukushima Medical University, Fukishama, Japan.

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http://dx.doi.org/10.1111/ced.14279DOI Listing

Autoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome.

Respirol Case Rep 2020 Jul 5;8(5):e00569. Epub 2020 May 5.

Eastern Health Melbourne VIC Australia.

We report the first case of autoimmune pulmonary alveolar proteinosis (PAP) associated with and preceding myelodysplastic syndrome. A 74-year-old female with a history of polymyalgia rheumatica presented with six months history of progressive exertional breathlessness. Examination revealed bilateral chest crackles and exertional desaturation. Read More

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http://dx.doi.org/10.1002/rcr2.569DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200416PMC

Pulmonary alveolar proteinosis after lung transplantation.

Respirol Case Rep 2020 Jul 5;8(5):e00566. Epub 2020 May 5.

QLD Lung Transplant Service The Prince Charles Hospital Brisbane QLD Australia.

We report the case of a 69-year-old man five-month post double lung transplant for idiopathic pulmonary fibrosis (IPF) who presented with progressive breathlessness, loss of lung function, and diffuse ground glass shadowing on the chest computed tomography. Transbronchial lung biopsy revealed foamy macrophages, hyperplasia of type II pneumocytes, and eosinophilic material in the alveolar space. Video thoracic lung biopsy was performed, and histology confirmed pulmonary alveolar proteinosis. Read More

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http://dx.doi.org/10.1002/rcr2.566DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7199162PMC

Reversible sirolimus-induced pulmonary alveolar proteinosis in a renal transplant patient.

Lung India 2020 May-Jun;37(3):252-256

Departamento De Ciencias Clinicas, Tecnológico De Monterrey, Escuela De Medicina Y Ciencias De La Salud, Dr. Ignacio Morones Prieto; Departamento de Cirugía, Hospital SNTE Sección 50; Departamento De Ciencias Clinicas, Tecnologico De Monterrey, Escuela Nacional De Ingeniería, Hospital Zambrano Hellion, Monterrey, Mexico.

Pulmonary alveolar proteinosis (PAP) is characterized by accumulation of surfactant-like lipoprotein material within distal bronchioles and alveoli due to impaired clearance. Clinically, PAP presents with dyspnea and cough. A 58-year-old Hispanic man presented with 6 months of productive cough, weight loss, and progressively worsening dyspnea. Read More

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http://dx.doi.org/10.4103/lungindia.lungindia_484_19DOI Listing

Pulmonary alveolar proteinosis.

Respirology 2020 May 3. Epub 2020 May 3.

Department of Radiology, CHU Rennes, Univ Rennes, Rennes, France.

PAP is an ultra-rare disease in which surfactant components, that impair gas exchange, accumulate in the alveolae. There are three types of PAP. The most frequent form, primary PAP, includes autoimmune PAP which accounts for over 90% of all PAP, defined by the presence of circulating anti-GM-CSF antibodies. Read More

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http://dx.doi.org/10.1111/resp.13831DOI Listing

Pulmonary Alveolar Proteinosis in a Patient with Systemic Lupus Erythematosus.

J Rheumatol 2020 May;47(5):779-780

Complexo Hospitalario A Coruña, Departments of Rheumatology, Pneumology, and Pathological Anatomy, Complexo Hospitalario Universitario A Coruña, A Coruña, Spain.

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http://dx.doi.org/10.3899/jrheum.181410DOI Listing

A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features.

Kurume Med J 2020 Jul 24;66(1):59-63. Epub 2020 Apr 24.

Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine.

A 78-year-old man who had worked in the building industry visited our hospital because of groundglass opacity with smoothly thickened, intralobular interstitial lines and interlobular septal lines on chest high-resolution computed tomography (HRCT). HRCT image also showed a focal area of reticulation and pleural thickening. Lung specimens obtained by surgical lung biopsy showed accumulations of intra-alveolar periodic acid-Schiffpositive materials, usual interstitial pneumonia (UIP)-like subpleural lung fibrosis and asbestos bodies (1 body/cm2 in high-power field, ×400). Read More

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http://dx.doi.org/10.2739/kurumemedj.MS661005DOI Listing

Longitudinal lung involvement of systemic lupus erythematosus-related vasculitis and alveolar proteinosis-like reaction.

Respirol Case Rep 2020 Jul 17;8(5):e00559. Epub 2020 Apr 17.

Second Division, Department of Internal Medicine Hamamatsu University School of Medicine Hamamatsu Japan.

A 44-year-old woman with no symptoms was referred to our hospital for thorough examination of consolidation opacity on her left lung, which was growing for four years. She was diagnosed with systemic lupus erythematosus (SLE) at the age of 35 years and had been treated with prednisolone 10 mg/day. Physical examination and bronchoscopy revealed no abnormality including microbiological tests. Read More

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http://dx.doi.org/10.1002/rcr2.559DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7163719PMC

Pulmonary Alveolar Proteinosis Syndrome.

Semin Respir Crit Care Med 2020 Apr 12;41(2):288-298. Epub 2020 Apr 12.

Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland.

Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by progressive accumulation of pulmonary surfactant. This results in dyspnea, secondary pulmonary and systemic infection, and in some cases respiratory failure. PAP syndrome occurs in distinct diseases, classified according to pathogenetic mechanism; these include primary PAP (due to disruption of granulocyte-macrophage colony-stimulating factor [GM-CSF] signaling), secondary PAP (due to reduction in alveolar macrophage numbers/functions), and congenital PAP (due to disruption of surfactant production). Read More

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http://dx.doi.org/10.1055/s-0039-3402727DOI Listing

Autoimmune pulmonary alveolar proteinosis developed during immunosuppressive treatment in polymyositis with interstitial lung disease: a case report.

BMC Pulm Med 2020 Apr 6;20(1):84. Epub 2020 Apr 6.

Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Minami-Uonuma, Japan.

Background: Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of surfactant proteins within the alveolar spaces. Autoimmune PAP (APAP) caused by elevated levels of GM-CSF autoantibodies (GM-Ab) is very rarely associated with systemic autoimmune disease. Here we report a case of APAP manifested during immunosuppressive treatment for polymyositis with interstitial lung disease. Read More

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http://dx.doi.org/10.1186/s12890-020-1110-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7132862PMC

Aluminium toxicosis: a review of toxic actions and effects.

Interdiscip Toxicol 2019 Oct 20;12(2):45-70. Epub 2020 Feb 20.

Department Veterinary Physiology and Biochemistry, Faculty of Veterinary Medicine, University of Maiduguri, Maiduguri, Nigeria.

Aluminium (Al) is frequently accessible to animal and human populations to the extent that intoxications may occur. Intake of Al is by inhalation of aerosols or particles, ingestion of food, water and medicaments, skin contact, vaccination, dialysis and infusions. Toxic actions of Al induce oxidative stress, immunologic alterations, genotoxicity, pro-inflammatory effect, peptide denaturation or transformation, enzymatic dysfunction, metabolic derangement, amyloidogenesis, membrane perturbation, iron dyshomeostasis, apoptosis, necrosis and dysplasia. Read More

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http://dx.doi.org/10.2478/intox-2019-0007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7071840PMC
October 2019

Proteogenomic analysis of granulocyte macrophage colony- stimulating factor autoantibodies in the blood of a patient with autoimmune pulmonary alveolar proteinosis.

Sci Rep 2020 Mar 18;10(1):4923. Epub 2020 Mar 18.

Niigata University Medical & Dental Hospital, Niigata, Japan.

Recently, attempts to reveal the structures of autoantibodies comprehensively using improved proteogenomics technology, have become popular. This technology identifies peptides in highly purified antibodies by using an Orbitrap device to compare spectra from liquid chromatography-tandem mass spectrometry against a cDNA database obtained through next-generation sequencing. In this study, we first analyzed granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies in a patient with autoimmune pulmonary alveolar proteinosis, using the trapped ion mobility spectrometry coupled with quadrupole time-of-flight (TIMS-TOF) instrument. Read More

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http://dx.doi.org/10.1038/s41598-020-61934-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7080758PMC

How to do whole lung lavage for treatment of pulmonary alveolar proteinosis.

ANZ J Surg 2020 05 26;90(5):877-878. Epub 2020 Feb 26.

Department of Cardiothoracic Surgery, Royal Adelaide Hospital, Adelaide, South Australia, Australia.

Pulmonary alveolar proteinosis is a rare condition characterized by progressive accumulation of surfactant lipoproteins in the alveolar space, leading to poor gas exchange. We describe our technique of whole lung lavage for management of pulmonary alveolar proteinosis. Read More

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http://dx.doi.org/10.1111/ans.15769DOI Listing

Natural Autoantibodies in Chronic Pulmonary Diseases.

Int J Mol Sci 2020 Feb 8;21(3). Epub 2020 Feb 8.

Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center, 5-1-1 Toneyama Toyonaka, Osaka 5600045, Japan.

In autoantibody-mediated autoimmune diseases, pathogenic autoantibodies generated by a failure of central or peripheral tolerance, have different effects mediated by a variety of mechanisms. Interestingly, even non-autoimmune chronic diseases have a set of disease-specific natural autoantibodies that are maintained for a long time. Because most of these natural autoantibodies target intracellular proteins or long non-coding RNAs, they are speculated to be non-pathological and have some important as yet unrecognized physiological functions such as debris clearance. Read More

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http://dx.doi.org/10.3390/ijms21031138DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7037933PMC
February 2020

Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study.

BMC Pulm Med 2020 Feb 6;20(1):34. Epub 2020 Feb 6.

Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-Ro 43-Gil, Songpa-Gu, Seoul, 05505, Republic of Korea.

Background: Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the Korean Health Insurance Review and Assessment service.

Methods: Data were extracted for adults who visited any secondary or tertiary medical institute between 2010 and 2016 with the PAP-related Korean Classification of Disease, 7th edition code J84. Read More

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http://dx.doi.org/10.1186/s12890-020-1074-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7006195PMC
February 2020
2.489 Impact Factor

Validation of a new serum granulocyte-macrophage colony-stimulating factor autoantibody testing kit.

ERJ Open Res 2020 Jan 27;6(1). Epub 2020 Jan 27.

Clinical and Translational Research Center, Niigata University Medical and Dental Hospital, Niigata, Japan.

Very recently, a modest but significant efficacy of granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation therapy for the treatment of mild to moderate autoimmune pulmonary alveolar proteinosis (aPAP) has been reported. As the ability to measure the level of GM-CSF autoantibody (GMAb) in the serum is required to decide the indication for this therapy, we developed a high-performance GMAb testing kit for clinical use. As the kit succeeded in reducing nonspecific IgG binding to the ELISA plate, the predictive performance shown in the training study to discriminate aPAP patients from healthy subjects was perfect, providing a cut-off value of 1. Read More

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http://dx.doi.org/10.1183/23120541.00259-2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6983497PMC
January 2020

Effects of indium exposure on respiratory symptoms: a retrospective cohort study in Japanese workers using health checkup data.

PeerJ 2020 15;8:e8413. Epub 2020 Jan 15.

Center for Innovative Clinical Medicine, Okayama University Hospital, Okayama, Japan.

Background: Indium compounds are known health hazards for lung cancer and interstitial pneumonia. Furthermore, they are related to emphysema, alveolar proteinosis, and cholesterol granuloma. In Japan, laws were revised in 2013 to tighten regulations on indium exposure in workplaces. Read More

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http://dx.doi.org/10.7717/peerj.8413DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6969551PMC
January 2020

Improvement of native pulmonary alveolar proteinosis after contralateral single living-donor lobar lung transplantation: A case report.

Pediatr Transplant 2020 03 27;24(2):e13659. Epub 2020 Jan 27.

Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

PAP is a rare disease characterized by the accumulation of surfactant materials in the alveolar spaces due to the imbalance of surfactant homeostasis (production and clearance). We herein report a case of an 8-year-old girl who developed PAP after BMT from her mother for the treatment of DBA. The anemia was improved by BMT; however, respiratory dysfunction due to graft-versus-host disease gradually progressed. Read More

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http://dx.doi.org/10.1111/petr.13659DOI Listing
March 2020
1.630 Impact Factor

Crazy Paving in Pulmonary Alveolar Proteinosis.

N Engl J Med 2020 Jan;382(3):275

Stellenbosch University, Stellenbosch, South Africa

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http://dx.doi.org/10.1056/NEJMicm1908563DOI Listing
January 2020

Inhaled GM-CSF for Pulmonary Alveolar Proteinosis. Reply.

N Engl J Med 2020 01;382(2):198

Niigata University Medical and Dental Hospital, Niigata, Japan

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http://dx.doi.org/10.1056/NEJMc1914606DOI Listing
January 2020

Inhaled GM-CSF for Pulmonary Alveolar Proteinosis.

N Engl J Med 2020 01;382(2):197

National and Kapodistrian University of Athens, Athens, Greece

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http://dx.doi.org/10.1056/NEJMc1914606DOI Listing
January 2020

[Rare occupational respiratory diseases].

Med Pr 2020 Jan 10;71(1):89-104. Epub 2019 Dec 10.

Instytut Medycyny Pracy im. prof. J. Nofera / Nofer Institute of Occupational Medicine, Łódź, Poland (Klinika Chorób Zawodowych i Zdrowia Środowiskowego, Oddział Chorób Zawodowych / Clinic of Occupational Diseases and Toxicology, Department of Occupational Diseases).

This paper reviews rare occupational respiratory diseases with uncommon causes. Among others, it refers to the Ardystil syndrome characterized by the occurrence of organizing pneumonia in the textile printing sprayers, as a result of inhalation of substances used in aerographic textile printing. Furthermore, secondary pulmonary alveolar proteinosis due to exposure to chemical and organic dusts was described, and so was the occurrence of the "vineyard sprayer's lung" in farm workers in Portugal. Read More

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http://dx.doi.org/10.13075/mp.5893.00895DOI Listing
January 2020

Aggravation of atherosclerosis by pulmonary exposure to indium oxide nanoparticles.

Nanotoxicology 2020 04 7;14(3):355-371. Epub 2020 Jan 7.

Lab of Toxicology, Department of Medicinal Biotechnology, College of Health Sciences, Dong-A University, Busan, Republic of Korea.

The use of indium oxide (InO) and indium-metal hybrids for various applications, including the manufacture of batteries and liquid crystal displays, increases the chances of human exposure to InO via inhalation, especially in occupational settings. However, there is little information available on the toxic effects of InO nanoparticles (NPs) on secondary organs following pulmonary exposure. In this study, we evaluated the effect of InO NPs on atherosclerotic plaque formation and the related mechanisms after pulmonary exposure in low-density lipoprotein receptor knockout () mice. Read More

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http://dx.doi.org/10.1080/17435390.2019.1704590DOI Listing

[Whole lung lavage in an infant with pulmonary alveolar proteinosis and literature review].

Zhonghua Er Ke Za Zhi 2020 Jan;58(1):46-50

Department of Pediatric Intensive Care Unit, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, China.

To investigate the safety, feasibility and operation key points of whole lung lavage in infants with pulmonary alveolar proteinosis. The clinical manifestations, genetic screening, therapeutic interventions and outcome of an infant with pulmonary alveolar proteinosis complicated with respiratory failure who received whole lung lavage in November 2018 in Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine were reported. Websites including PubMed, Springer Link, China National Knowledge Infrastructure (CNKI), Weipu Database, and Wanfang Database were searched using the key words of "whole lung lavage" "pediatric" and "pulmonary alveolar proteinosis" for articles published from their establishments to April 2019. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.01.011DOI Listing
January 2020

Therapeutic effect of subcutaneous injection of low dose recombinant human granulocyte-macrophage colony-stimulating factor on pulmonary alveolar proteinosis.

Respir Res 2020 Jan 2;21(1). Epub 2020 Jan 2.

Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, 507 Zheng Min Road, Shanghai, 200433, China.

Objective: To observe the efficacy of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for pulmonary alveolar proteinosis (PAP).

Materials And Methods: A total of 55 patients with PAP were screened at Shanghai Pulmonary Hospital between May 2014 and May 2018. Among these, 42 were diagnosed with idiopathic PAP, 24 were included in this study, 20 were treated for 6 months, and 17 were followed up for additional 6 months. Read More

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http://dx.doi.org/10.1186/s12931-019-1261-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6941258PMC
January 2020

Fetal monocytes possess increased metabolic capacity and replace primitive macrophages in tissue macrophage development.

EMBO J 2020 Feb 2;39(3):e103205. Epub 2020 Jan 2.

Department of Biology, Institute of Molecular Health Sciences, ETH Zürich, Zürich, Switzerland.

Tissue-resident macrophages (MΦ ) originate from at least two distinct waves of erythro-myeloid progenitors (EMP) arising in the yolk sac (YS) at E7.5 and E8.5 with the latter going through a liver monocyte intermediate. Read More

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http://dx.doi.org/10.15252/embj.2019103205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996567PMC
February 2020

Knowledge of Secondary Pulmonary Alveolar Proteinosis Complicated with Myelodysplastic Syndrome.

Authors:
Haruyuki Ishii

Intern Med 2020 04 26;59(8):1019-1020. Epub 2019 Dec 26.

Department of Respiratory Medicine, Kyorin University School of Medicine, Japan.

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http://dx.doi.org/10.2169/internalmedicine.4193-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7205530PMC

Secondary Pulmonary Alveolar Proteinosis Following Treatment with Azacitidine for Myelodysplastic Syndrome.

Intern Med 2020 Apr 26;59(8):1081-1086. Epub 2019 Dec 26.

Department of Hematology, Nagasaki University Graduate School of Biomedical Sciences, Japan.

Secondary pulmonary alveolar proteinosis (sPAP) is a complication of myelodysplastic syndrome (MDS). A 60-year-old woman was diagnosed with MDS with excess blasts-1. Fifty-four months after the initial diagnosis, treatment with azacitidine was initiated. Read More

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http://dx.doi.org/10.2169/internalmedicine.3770-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7205539PMC

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

J Clin Invest 2020 Apr;130(4):1669-1682

Translational Autoinflammatory Diseases Section (TADS), NIAID/NIH, Bethesda, Maryland, USA.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing. Read More

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http://dx.doi.org/10.1172/JCI129301DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7108905PMC

Valuable Serum Markers in Pulmonary Alveolar Proteinosis.

Dis Markers 2019 11;2019:9709531. Epub 2019 Nov 11.

Department of Respiratory Medicine, Nanjing Drum Tower Hospital, Nanjing University Medical School, Nanjing 210008, China.

Objective: Several serum markers were reported to reflect the severity of pulmonary alveolar proteinosis (PAP). The aim of this study is to investigate a reliable and facile marker to access and monitor the clinical course of PAP in a large cohort.

Methods: PAP patients from January 2010 to June 2018 were enrolled. Read More

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http://dx.doi.org/10.1155/2019/9709531DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6885220PMC

The early onset and persistent worsening pulmonary alveolar proteinosis in rats by indium oxide nanoparticles.

Nanotoxicology 2020 05 27;14(4):468-478. Epub 2019 Nov 27.

Lab of Toxicology, Department of Medicinal Biotechnology, College of Health Sciences, Dong-A University, Busan, Republic of Korea.

Workplace inhalation exposure to indium compounds has been reported to produce 'indium lung disease' characterized by pulmonary alveolar proteinosis (PAP), granulomas, and pulmonary fibrosis. However, there is little information about the pulmonary toxicity of nano-sized indium oxide (InO), which is widely used in various applications such as liquid crystal displays. In this study, we evaluated the time-course and dose-dependent lung injuries by InO nanoparticles (NPs) after a single intratracheal instillation to rats. Read More

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http://dx.doi.org/10.1080/17435390.2019.1694184DOI Listing