2,016 results match your criteria Pulmonary Alveolar Proteinosis


Interstitial lung disease as a late complication of pulmonary alveolar proteinosis.

Radiol Case Rep 2019 May 1;14(5):572-575. Epub 2019 Mar 1.

McGill University Health Centre, Department of Diagnostic Radiology, Montreal General Hospital, 1650 Cedar Avenue, Montreal QC H3G 1A4, Canada.

Pulmonary alveolar proteinosis is a rare condition characterized by accumulation of intra-alveolar surfactant. Here, we report a case of interstitial lung disease which developed over the years in a patient with pulmonary alveolar proteinosis. Read More

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http://dx.doi.org/10.1016/j.radcr.2019.02.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402299PMC

Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report.

BMC Pulm Med 2019 Mar 12;19(1):61. Epub 2019 Mar 12.

Department of Medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Tripler AMC, Hawaii, 96859, USA.

Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Read More

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http://dx.doi.org/10.1186/s12890-019-0822-xDOI Listing
March 2019
1 Read

The influence of genetics on therapeutic developments in pulmonary alveolar proteinosis.

Authors:
Ilaria Campo

Curr Opin Pulm Med 2019 Mar 6. Epub 2019 Mar 6.

SC Pneumologia - Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Purpose Of Review: Pulmonary alveolar proteinosis (PAP) is characterized by the massive accumulation of lipoproteinaceous material within alveoli, which results in progressive respiratory failure. The abnormalities in surfactant clearance are caused by defective pulmonary macrophages, whose terminal differentiation is GM-CSF-dependent. In hereditary PAP, the rupture of GM-CSF signaling is because of mutations in the GM-CSF receptor genes. Read More

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http://dx.doi.org/10.1097/MCP.0000000000000576DOI Listing
March 2019
2 Reads

Pulmonary alveolar proteinosis.

Nat Rev Dis Primers 2019 Mar 7;5(1):16. Epub 2019 Mar 7.

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

Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive dyspnoea of insidious onset, hypoxaemic respiratory failure, secondary infections and pulmonary fibrosis. PAP can be classified into different types on the basis of the pathogenetic mechanism: primary PAP is characterized by the disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling and can be autoimmune (caused by elevated levels of GM-CSF autoantibodies) or hereditary (due to mutations in CSF2RA or CSF2RB, encoding GM-CSF receptor subunits); secondary PAP results from various underlying conditions; and congenital PAP is caused by mutations in genes involved in surfactant production. Read More

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http://dx.doi.org/10.1038/s41572-019-0066-3DOI Listing
March 2019
1 Read

Pulmonary alveolar proteinosis.

Authors:

Nat Rev Dis Primers 2019 Mar 7;5(1):17. Epub 2019 Mar 7.

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http://dx.doi.org/10.1038/s41572-019-0070-7DOI Listing
March 2019
1 Read

Pulmonary alveolar proteinosis: An autoimmune disease lacking an HLA association.

PLoS One 2019 7;14(3):e0213179. Epub 2019 Mar 7.

University of Colorado Anschutz Medical Campus, Department of Medicine, Aurora, CO, United States of America.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of pulmonary surfactant in alveolar macrophages and alveoli, resulting in respiratory impairment and an increased risk of opportunistic infections. Autoimmune PAP is an autoimmune lung disease that is caused by autoantibodies directed against granulocyte-macrophage colony-stimulating factor (GM-CSF). A shared feature among many autoimmune diseases is a distinct genetic association to HLA alleles. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213179PLOS
March 2019
7 Reads

Automated Parenchymal Pattern Analysis of Treatment Responses in Pulmonary Alveolar Proteinosis.

Am J Respir Crit Care Med 2019 Mar 1. Epub 2019 Mar 1.

Children's Hospital Medical Center, Division of Pulmonary Biology, University of Cincinnati, Cincinnati, Ohio, United States ;

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http://dx.doi.org/10.1164/rccm.201810-1918IMDOI Listing
March 2019
1 Read

The Clinical Significance of a 'Crazy-Paving' Pattern on Chest Radiology.

Eur J Case Rep Intern Med 2018 27;5(12):000978. Epub 2018 Dec 27.

Pulmonary Department, Loewenstein Lung Center, Löwenstein, Germany.

A crazy-paving pattern is a non-specific radiological sign which is characterized by the presence of diffuse ground-glass attenuation associated with interlobular septal thickening and intralobular lines. It was initially described as a pathognomonic sign of pulmonary alveolar proteinosis. However, it can be also found in many other diffuse acute and chronic lung diseases including diffuse alveolar haemorrhage (DAH), a rare and life-threatening clinical syndrome which can be caused by many conditions, the most frequent of these being capillaritis associated with systemic autoimmune diseases. Read More

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http://dx.doi.org/10.12890/2018_000978DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346976PMC
December 2018
11 Reads

Whole-lung lavage for severe pulmonary alveolar proteinosis assisted by veno-venous extracorporeal membrane oxygenation: a case report.

Can J Respir Ther 2018 Dec 5;55(1). Epub 2018 Dec 5.

Department of Anesthesiology, Centro Hospitalar São João, Porto, Portugal.

Pulmonary alveolar proteinosis (PAP) is a rare pathology characterized by accumulation of phospholipoproteinaceous material within the alveoli. The evolution of PAP is variable and treatment modalities are limited. Pharmacological therapeutic targets are being actively developed, but whole-lung lavage (WLL), first described in the 1960s, remains the cornerstone of therapy. Read More

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http://dx.doi.org/10.29390/cjrt-2018-019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345579PMC
December 2018
6 Reads

Avian pulmonary proteinosis: six cases and a review of the literature.

J Vet Diagn Invest 2019 Feb 8:1040638719830753. Epub 2019 Feb 8.

California Animal Health and Food Safety Laboratory System, School of Veterinary Medicine, University of California, Davis, CA.

Pulmonary alveolar proteinosis (PAP) is a disease of surfactant clearance in which functional abnormalities in alveolar macrophages lead to accumulation of surfactant within alveoli in mammals. Histologic examination of 6 avian autopsies, including 4 chickens, a turkey, and a cockatiel, revealed accumulation of hypereosinophilic densely arrayed lamellar material in the lungs that was magenta by periodic acid-Schiff stain and diastase resistant. Transmission electron microscopy of the proteinaceous material in 2 cases demonstrated alternating electron-dense and electron-lucent lamellae that formed whorls and had a regular periodicity of 6-14 nm, consistent with pulmonary surfactant. Read More

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http://dx.doi.org/10.1177/1040638719830753DOI Listing
February 2019
1 Read

Blood Testing for Differential Diagnosis of Pulmonary Alveolar Proteinosis Syndrome.

Chest 2019 Feb;155(2):450-452

Translational Pulmonary Science Center, Cincinnati, OH; Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, OH; Division of Pulmonary Medicine, Children's Hospital Medical Center, Cincinnati, OH; Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati College of Medicine, Cincinnati, OH. Electronic address:

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http://dx.doi.org/10.1016/j.chest.2018.11.002DOI Listing
February 2019
1 Read

Methionyl-tRNA synthetase novel mutation causes pulmonary alveolar proteinosis.

Saudi Med J 2019 Feb;40(2):195-198

Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia. E-mail.

The  methionyl-tRNA  synthetase  (MARS)  mutation is  a  very  rare  cause  of  congenital  pulmonary  alveolar proteinosis.We report a 6-month-old boy born with symmetrical intrauterine growth retardation presented with unexplained persistent tachypnea and hypoxemia associated with severe failure to thrive, anemia, hypoalbuminemia and hepatomegaly. Detailed pulmonary investigations including computed tomography chest scan, bronchoscopy and bronchoalveolar lavage revealed pulmonary alveolar proteinosis. Read More

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https://smj.org.sa/index.php/smj/article/view/smj.2019.2.239
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http://dx.doi.org/10.15537/smj.2019.2.23908DOI Listing
February 2019
5 Reads

Atypical alveolar proteinosis.

Indian J Radiol Imaging 2018 Oct-Dec;28(4):439-441

Department of Medical Imaging Services of JRA Hospital, Antananarivo, Madagascar.

Alveolar proteinosis is a rare pulmonary disease characterized by intra-alveolar accumulation of surfactant composed of lipoproteinaceous material, related to a lack of surfactant resorption by alveolar macrophages. Crazy paving pattern is characteristic, but not specific. The multinodular forms of this affection remain exceptional. Read More

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http://dx.doi.org/10.4103/ijri.IJRI_170_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319097PMC
January 2019
2 Reads

ADAR1 Is Required for Dendritic Cell Subset Homeostasis and Alveolar Macrophage Function.

J Immunol 2019 Feb 16;202(4):1099-1111. Epub 2019 Jan 16.

Department of Transfusion Medicine and Hemostaseology, University Hospital Erlangen, Erlangen 91054, Germany;

RNA editing by adenosine deaminases acting on dsRNA (ADAR) has become of increasing medical relevance, particularly because aberrant ADAR1 activity has been associated with autoimmunity and malignancies. However, the role of ADAR1 in dendritic cells (DC), representing critical professional APCs, is unknown. We have established conditional murine CD11c Cre-mediated ADAR1 gene ablation, which did not induce general apoptosis in CD11c cells but instead manifests in cell type-specific effects in DC subpopulations. Read More

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http://dx.doi.org/10.4049/jimmunol.1800269DOI Listing
February 2019
1 Read

Myeloid malignancies with somatic GATA2 mutations can be associated with an immunodeficiency phenotype.

Leuk Lymphoma 2019 Jan 16:1-9. Epub 2019 Jan 16.

a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.

Germline mutations in GATA2 are associated with a complex immunodeficiency and cancer predisposition syndrome. Somatic GATA2 in myeloid malignancies may impart a similar phenotype. We reviewed adult patients with a diagnosis of GATA2 hematological malignancy who were referred to our HHMC for genetic testing, and identified to have somatic GATA2. Read More

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https://www.tandfonline.com/doi/full/10.1080/10428194.2018.1
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http://dx.doi.org/10.1080/10428194.2018.1551535DOI Listing
January 2019
7 Reads

GATA2 deficiency and human hematopoietic development modeled using induced pluripotent stem cells.

Blood Adv 2018 Dec;2(23):3553-3565

Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health (NIH), Bethesda, MD.

GATA2 deficiency is an inherited or sporadic genetic disorder characterized by distinct cellular deficiency, bone marrow failure, various infections, lymphedema, pulmonary alveolar proteinosis, and predisposition to myeloid malignancies resulting from heterozygous loss-of-function mutations in the gene. How heterozygous mutations affect human hematopoietic development or cause characteristic cellular deficiency and eventual hypoplastic myelodysplastic syndrome or leukemia is not fully understood. We used induced pluripotent stem cells (iPSCs) to study hematopoietic development in the setting of GATA2 deficiency. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018017137DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290105PMC
December 2018
8 Reads

[Crazy Paving Pattern of the Lung].

Pneumologie 2019 Jan 7;73(1):49-53. Epub 2018 Dec 7.

Abteilung für Pneumologie, Fachklinik für Lungenerkrankungen Donaustauf, Donaustauf.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease. PAP results from impaired surfactant clearance. In adults, autoimmune pulmonary alveolar proteinosis is present in 90 - 95 % of the cases. Read More

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http://dx.doi.org/10.1055/a-0767-7960DOI Listing
January 2019
1 Read

Pulmonary alveolar proteinosis - a crazy presentation of dyspnea.

Eur Clin Respir J 2019 4;6(1):1552065. Epub 2018 Dec 4.

Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.

This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a. Read More

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https://www.tandfonline.com/doi/full/10.1080/20018525.2018.1
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http://dx.doi.org/10.1080/20018525.2018.1552065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282426PMC
December 2018
13 Reads

CT findings in pulmonary alveolar proteinosis: serial changes and prognostic implications.

J Thorac Dis 2018 Oct;10(10):5774-5783

Department of Radiology, Samsung Medical Center, Seoul, Korea.

Background: Pulmonary alveolar proteinosis (PAP) is a rare disease of unknown origin, characterized by impaired surfactant metabolism. In some patients, residual disease is observed after treatment; notably, this may progress to end-stage pulmonary fibrosis. This study was performed to evaluate changes in lung abnormalities on serial CT scans performed on the lungs of patients diagnosed with PAP, as well as to identify factors that can be used to predict clinical improvement in PAP. Read More

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http://dx.doi.org/10.21037/jtd.2018.09.86DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236152PMC
October 2018
2 Reads

Blood testing in the diagnosis of pulmonary alveolar proteinosis - Authors' reply.

Lancet Respir Med 2018 11;6(11):e55

Department of Pulmonary Medicine, Respiratory Institute, and Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA.

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http://dx.doi.org/10.1016/S2213-2600(18)30373-4DOI Listing
November 2018
1 Read

Blood testing in the diagnosis of pulmonary alveolar proteinosis.

Lancet Respir Med 2018 11;6(11):e54

Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA; Translational Pulmonary Science Center, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA; Division of Pulmonary Medicine, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA; Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA. Electronic address:

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http://dx.doi.org/10.1016/S2213-2600(18)30372-2DOI Listing
November 2018

Autoimmune pulmonary alveolar proteinosis presenting peripheral ground-glass opacities.

Respirol Case Rep 2019 Jan 13;7(1):e00385. Epub 2018 Nov 13.

Department of Respiratory Medicine Jizankai Medical Fundation Tsuboi Cancer Center Hospital Koriyama Japan.

Autoimmune pulmonary alveolar proteinosis should be considered in the differential diagnosis of peripheral ground-glass opacities. Read More

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http://dx.doi.org/10.1002/rcr2.385DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232946PMC
January 2019
3 Reads

Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study.

Orphanet J Rare Dis 2018 10 20;13(1):182. Epub 2018 Oct 20.

Clinical Research Centre, National Hospital Organization Kinki-Chuo Chest Medical Centre, 1180 Nagasone-cho, Kita-ku, Sakai City, Osaka, 591-8555, Japan.

Background: Lymphangioleiomyomatosis (LAM) and connective tissue diseases (CTDs) occur more frequently among women than men. We investigated the frequency of comorbid CTD and positive serum autoantibody findings in patients with LAM.

Methods: A total of 152 patients with LAM were prospectively and consecutively registered in the National Hospital Organization Kinki-Chuo Chest Medical Centre cohort. Read More

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http://dx.doi.org/10.1186/s13023-018-0933-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6195983PMC
October 2018
4 Reads

Secondary pulmonary alveolar proteinosis in acute myeloid leukemia.

QJM 2018 Oct 8. Epub 2018 Oct 8.

Department of Molecular and Internal Medicine, Institute of Biomedical & Health Sciences, Hiroshima University, Minami-ku, Hiroshima, Japan.

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https://academic.oup.com/qjmed/advance-article/doi/10.1093/q
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http://dx.doi.org/10.1093/qjmed/hcy224DOI Listing
October 2018
5 Reads

Novel methionyl-tRNA synthetase gene variants/phenotypes in interstitial lung and liver disease: A case report and review of literature.

World J Gastroenterol 2018 Sep;24(36):4208-4216

Department of Hepatology, Children's Hospital of Fudan University, Shanghai 201102, China.

Interstitial lung and liver disease (ILLD) is caused by biallelic mutations in the methionyl-tRNA synthetase () gene. To date, no genetic changes other than missense variants were reported in the literature. Here, we report a five-month old female infant with typical ILLD (failure to thrive, developmental delay, jaundice, diffuse interstitial lung disease, hepatomegaly with severe steatosis, anemia, and thrombocytosis) showing novel phenotypes such as kidney stones, acetabular dysplasia, prolonged fever, and extreme leukocytosis. Read More

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http://dx.doi.org/10.3748/wjg.v24.i36.4208DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6158480PMC
September 2018
5 Reads

Better approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses.

Respir Res 2018 Aug 31;19(1):163. Epub 2018 Aug 31.

Department of Respiratory and Critical Care Medicine, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, No.1095, Jie Fang Road, Han Kou District, Wu Han, 430030, Hu Bei Province, China.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare pulmonary disease caused by functional deficiency of granulocyte-macrophage colony-stimulating factor (GM-CSF). GM-CSF therapy in aPAP has been reported effective in some studies. This meta-analyses aimed to evaluate whether GM-CSF therapy, including inhaled and subcutaneous GM-CSF have therapeutic effect in aPAP patients. Read More

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http://dx.doi.org/10.1186/s12931-018-0862-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117963PMC
August 2018
2 Reads

Pulmonary Alveolar Proteinosis in Hereditary and Autoimmune Forms With 2 Cases.

Pediatr Emerg Care 2018 Aug 14. Epub 2018 Aug 14.

Department of Pathology, Dokuz Eylul University, Izmir, Turkey.

Pulmonary alveolar proteinosis (PAP) is a respiratory pathology characterized by the accumulation and increase of surfactant-derived material in the lungs. In clinical practice, PAP may present as the primary form, which includes autoimmune and hereditary PAP, or as the secondary form. Diffuse alveolar radiopacities on chest x-ray and the crazy-paving pattern on high-resolution computed tomography are important, although not specific findings for PAP. Read More

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http://Insights.ovid.com/crossref?an=00006565-900000000-9835
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http://dx.doi.org/10.1097/PEC.0000000000001536DOI Listing
August 2018
14 Reads

Von Hippel-Lindau Protein Is Required for Optimal Alveolar Macrophage Terminal Differentiation, Self-Renewal, and Function.

Cell Rep 2018 Aug;24(7):1738-1746

Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), Madrid 28029, Spain. Electronic address:

The rapid transit from hypoxia to normoxia in the lung that follows the first breath in newborn mice coincides with alveolar macrophage (AM) differentiation. However, whether sensing of oxygen affects AM maturation and function has not been previously explored. We have generated mice whose AMs show a deficient ability to sense oxygen after birth by deleting Vhl, a negative regulator of HIF transcription factors, in the CD11c compartment (CD11cΔVhl mice). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22111247183112
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http://dx.doi.org/10.1016/j.celrep.2018.07.034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6113928PMC
August 2018
4 Reads

iPSC-Derived Macrophages Effectively Treat Pulmonary Alveolar Proteinosis in Csf2rb-Deficient Mice.

Stem Cell Reports 2018 Sep 9;11(3):696-710. Epub 2018 Aug 9.

Research Group Reprogramming and Gene Therapy, Hannover Medical School (MHH), Hannover, Germany; Institute of Experimental Hematology, MHH, Hannover Medical School, Carl-Neuberg-Str.1, 30625 Hannover, Germany; Cluster of Excellence REBIRTH, MHH, Hannover, Germany.

Induced pluripotent stem cell (iPSC)-derived hematopoietic cells represent a highly attractive source for cell and gene therapy. Given the longevity, plasticity, and self-renewal potential of distinct macrophage subpopulations, iPSC-derived macrophages (iPSC-Mφ) appear of particular interest in this context. We here evaluated the airway residence, plasticity, and therapeutic efficacy of iPSC-Mφ in a murine model of hereditary pulmonary alveolar proteinosis (herPAP). Read More

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http://dx.doi.org/10.1016/j.stemcr.2018.07.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6135208PMC
September 2018
19 Reads

Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis.

Nat Commun 2018 08 7;9(1):3127. Epub 2018 Aug 7.

Translational Pulmonary Science Center, Children's Hospital Medical Center, Cincinnati, OH, USA.

Pulmonary alveolar proteinosis (PAP) is a syndrome of reduced GM-CSF-dependent, macrophage-mediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown. Here, we examine the lipids accumulating in alveolar macrophages and surfactant to define the pathogenesis of PAP and evaluate a novel pharmacotherapeutic approach. In PAP patients, alveolar macrophages have a marked increase in cholesterol but only a minor increase in phospholipids, and pulmonary surfactant has an increase in the ratio of cholesterol to phospholipids. Read More

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http://dx.doi.org/10.1038/s41467-018-05491-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081448PMC
August 2018
22 Reads

Disseminated Cryptococcus gattii infection preceding onset of pulmonary alveolar proteinosis.

Respirol Case Rep 2018 Oct 2;6(7):e00357. Epub 2018 Aug 2.

Department of Respiratory and Critical Care Medicine Changi General Hospital Singapore.

A 50-year-old immunocompetent man presented with intracranial space-occupying lesions and a right lung mass. This was found to be disseminated Cryptococcus gattii infection. Following 15 months of anti-fungal therapy, imaging showed reduction in the size of the pulmonary cryptococcoma and new multi-lobar ground-glass opacities interspersed with a crazy-paving pattern. Read More

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http://dx.doi.org/10.1002/rcr2.357DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6071436PMC
October 2018
2 Reads

Prevalence and healthcare burden of pulmonary alveolar proteinosis.

Orphanet J Rare Dis 2018 07 31;13(1):129. Epub 2018 Jul 31.

Translational Pulmonary Science Center, Children's Hospital Medical Center, Cincinnati, OH, USA.

Pulmonary alveolar proteinosis (PAP) is a rare syndrome of alveolar surfactant accumulation, resulting hypoxemic respiratory failure, and increased infection risk. Despite advances in our understanding of disease pathogenesis and the availability of improved diagnostics, the epidemiology and healthcare burden of PAP remain poorly defined. To determine the prevalence, and healthcare utilization and costs associated with PAP, we interrogated a large health insurance claims database containing comprehensive data for approximately 15 million patients in the United States. Read More

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http://dx.doi.org/10.1186/s13023-018-0846-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6069872PMC
July 2018
23 Reads

The presence of autoimmune antibodies in pulmonary alveolar proteinosis does not necessarily imply idiopathic disease.

Lancet Respir Med 2018 09 26;6(9):e48. Epub 2018 Jul 26.

Department of Public Health and Primary Care, Centre for Environment and Health, KU Leuven, B-3000 Leuven, Belgium.

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http://dx.doi.org/10.1016/S2213-2600(18)30299-6DOI Listing
September 2018
3 Reads

Clinical and molecular features of X-linked hyper IgM syndrome - An experience from North India.

Clin Immunol 2018 Oct 25;195:59-66. Epub 2018 Jul 25.

Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

X-linked hyper IgM Syndrome (XLHIGM), the most frequent form of the Hyper IgM syndromes is a primary immune deficiency resulting from a mutation in the CD40 ligand gene (CD40LG). We analyzed the clinical and laboratory features of ten patients with XLHIGM, who were diagnosed at a tertiary care hospital in North India. Most common infections were sinopulmonary infections (80%) and diarrhea (50%). Read More

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http://dx.doi.org/10.1016/j.clim.2018.07.013DOI Listing
October 2018
15 Reads
3.672 Impact Factor

Germline GATA2 Mutation and Bone Marrow Failure.

Hematol Oncol Clin North Am 2018 Aug 28;32(4):713-728. Epub 2018 May 28.

Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA.

GATA2 deficiency is an immunodeficiency and bone marrow failure disorder caused by pathogenic variants in GATA2. It is inherited in an autosomal-dominant pattern or can be due to de novo sporadic germline mutation. Patients commonly have B-cell, dendritic cell, natural killer cell, and monocytopenias, and are predisposed to myelodysplastic syndrome, acute myeloid leukemia, and chronic myelomonocytic leukemia. Read More

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http://dx.doi.org/10.1016/j.hoc.2018.04.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6128284PMC
August 2018
4 Reads

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series.

J Bras Pneumol 2018 May-Jun;44(3):231-236

. Divisão de Pneumologia, Instituto do Coração - InCor - Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP) Brasil.

Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Read More

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http://dx.doi.org/10.1590/S1806-37562017000000168DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6188683PMC
February 2019
5 Reads

Pulmonary alveolar proteinosis in children: An unusual presentation with significant clinical impact.

Indian J Pathol Microbiol 2018 Jul-Sep;61(3):418-420

Department of Pathology, Anand Diagnostic Laboratory, Bengaluru, Karnataka, India.

Pulmonary alveolar proteinosis (PAP) - an unusual diffuse lung disease characterized by alveolar accumulation of phospholipoprotein material, with a peak incidence in third to fourth decade and male predominance is also described in children. Recorded prevalence is 0.1/100,000 individuals. Read More

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http://dx.doi.org/10.4103/IJPM.IJPM_17_17DOI Listing
November 2018
15 Reads

Assay system development to measure the concentration of sargramostim with high specificity in patients with autoimmune pulmonary alveolar proteinosis after single-dose inhalation.

J Immunol Methods 2018 Sep 9;460:1-9. Epub 2018 Jul 9.

Bioscience Medical Research Center, Niigata University Medical & Dental Hospital Niigata, Japan; Clinical and Translational Research Center, Niigata University Medical & Dental Hospital Niigata, Japan. Electronic address:

During a clinical trial of a Saccharomyces cerviciae-derived recombinant human granulocyte-macrophage colony stimulating factor (rhGM-CSF), sargramostim, in patients with autoimmune pulmonary alveolar proteinosis (aPAP), we conducted a pharmacokinetic study of single-dose sargramostim inhalation. Several problems were encountered whereby sargramostim formed an immune-complex with GM-CSF autoantibodies (GMAbs) immediately after entering the body; thus, we could not measure the concentration of sargramostim using a commercial high sensitivity enzyme-linked immunosorbent assay (ELISA). Moreover, the ELISA could not discriminate inhaled sargramostim from intrinsic GM-CSF. Read More

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http://dx.doi.org/10.1016/j.jim.2018.05.012DOI Listing
September 2018
4 Reads

Secondary pulmonary alveolar proteinosis predominant in the transplanted lung in patients with idiopathic interstitial pneumonia: an autopsy case.

J Thorac Dis 2018 May;10(5):E351-E354

Department of Respiratory Medicine, Yokohama, Kanagawa Cardiovascular and Respiratory Center, Japan.

A man in his 40 s with idiopathic interstitial pneumonia underwent cadaveric left single-lung transplantation from a brain-dead donor in October 2014. In October 2015, chest high-resolution computed tomography revealed centrilobular ground-glass opacities (GGOs) predominantly in the transplanted left lung, and subsequently, the shadows progressed to a geographic GGO without crazy paving. Bronchoalveolar lavage fluid analysis revealed an opaque and milky appearance, and cytopathology demonstrated foamy alveolar macrophages and abundant granular, acellular, eosinophilic, and amorphous material in the background. Read More

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http://dx.doi.org/10.21037/jtd.2018.04.49DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6006133PMC
May 2018
10 Reads

Monitoring whole-lung lavage using lung ultrasound: The changing phases of the lung.

Lung India 2018 Jul-Aug;35(4):350-353

Department of Pulmonary Medicine, St. John's National Academy of Health Sciences, Bengaluru, Karnataka, India.

Lung ultrasound (LUS) has been proven to yield valuable information for lung and pleural pathology. It is well validated for assessing extravascular lung water. It can also be used to monitor stages of controlled lung de-aeration in whole lung lavage (WLL) which is the treatment for Pulmonary Alveolar Protienosis (PAP),characterized by abnormal surfactant in the alveoli affecting gas exchange . Read More

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http://dx.doi.org/10.4103/lungindia.lungindia_344_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6034381PMC
July 2018
19 Reads

The mechanism of GM-CSF inhibition by human GM-CSF auto-antibodies suggests novel therapeutic opportunities.

MAbs 2018 10 12;10(7):1018-1029. Epub 2018 Sep 12.

a St. Vincent's Institute of Medical Research , Australian Cancer Research Foundation Rational Drug Discovery Centre , Fitzroy , Victoria , Australia.

Granulocyte-macrophage colony-stimulating factor (GM-CSF) is a hematopoietic growth factor that can stimulate a variety of cells, but its overexpression leads to excessive production and activation of granulocytes and macrophages with many pathogenic effects. This cytokine is a therapeutic target in inflammatory diseases, and several anti-GM-CSF antibodies have advanced to Phase 2 clinical trials in patients with such diseases, e.g. Read More

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http://dx.doi.org/10.1080/19420862.2018.1494107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204844PMC
October 2018
10 Reads

Novel application of cryobiopsy in the diagnosis of pulmonary alveolar proteinosis.

Respirol Case Rep 2018 Aug 20;6(6):e00336. Epub 2018 Jun 20.

School of Medicine China Medical University Taichung Taiwan.

Pulmonary alveolar proteinosis (PAP) is a rare disease diagnosed pathologically by the build up of surfactant in the alveolar spaces. Establishing a diagnosis usually requires invasive procedures such as bronchoalveolar lavage and forceps biopsy to obtain tissue specimens. Infrequently, surgery is required when histopathological results from other modalities are equivocal. Read More

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http://dx.doi.org/10.1002/rcr2.336DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6010806PMC
August 2018
3 Reads

Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication.

J Thorac Dis 2018 Apr;10(4):2467-2473

Department of Pneumonology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Whole lung lavage (WLL) under general anesthesia with a double-lumen endobronchial intubation has remained standard treatment option for pulmonary alveolar proteinosis (PAP) for over fifty years now. To the best of our knowledge, this is the first description of noninvasive ventilation (NIV) as an innovative alternative, which enables safe and effective treatment. NIV support enabled cost-effective lavage of the most affected segments and resulted in restoration and long-term maintenance of exercise capacity and diffusion, without WLL related hypoxaemia, volume overload, intubation, or mechanical ventilation related complications. Read More

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http://dx.doi.org/10.21037/jtd.2018.04.12DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5949484PMC
April 2018
6 Reads

Mutations in MARS identified in a specific type of pulmonary alveolar proteinosis alter methionyl-tRNA synthetase activity.

FEBS J 2018 Jul 25;285(14):2654-2661. Epub 2018 May 25.

Institute for Integrative Biology of the Cell (I2BC), CEA, CNRS, Univ. Paris-Sud, Université Paris-Saclay, Gif-sur-Yvette, France.

Biallelic missense mutations in MARS are responsible for rare but severe cases of pulmonary alveolar proteinosis (PAP) prevalent on the island of La Réunion. MARS encodes cytosolic methionyl-tRNA synthetase (MetRS), an essential translation factor. The multisystemic effects observed in patients with this form of PAP are consistent with a loss-of-function defect in an ubiquitously expressed enzyme. Read More

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http://dx.doi.org/10.1111/febs.14510DOI Listing
July 2018
4 Reads

PPAR-γ in innate and adaptive lung immunity.

J Leukoc Biol 2018 Oct 16;104(4):737-741. Epub 2018 May 16.

Department of Biology, Institute of Molecular Health Sciences, Zurich, Switzerland.

The transcription factor PPAR-γ (peroxisome proliferator-activated receptor-γ) is a key regulator of lung immunity exhibiting multiple cell type specific roles in controlling development and function of the lung immune system. It is strictly required for the generation of alveolar macrophages by controlling differentiation of fetal lung monocyte precursors. Furthermore, it plays an important role in lung allergic inflammation by licensing lung dendritic cell t helper 2 (Th2) priming capacity as well as acting as a master transcription factor for pathogenic Th2 cells. Read More

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http://doi.wiley.com/10.1002/JLB.3MR0118-034R
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http://dx.doi.org/10.1002/JLB.3MR0118-034RDOI Listing
October 2018
7 Reads

Autoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim).

Respir Med Case Rep 2018 23;23:167-169. Epub 2018 Feb 23.

Department of Respiratory Diseases and Allergology, Aarhus University Hospital, Denmark.

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare parenchymal lung disease characterized by accumulation of surfactant in the airways with high levels of granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies in blood. Disease leads to hypoxemic respiratory failure. Whole lung lavage (WLL) is considered the first line therapy, but procedure can be quite demanding, specifically for children. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.02.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5925949PMC
February 2018
8 Reads

Pulmonary Alveolar Proteinosis with Ulcerative Colitis.

Intern Med 2018 Sep 27;57(18):2705-2708. Epub 2018 Apr 27.

Department of Respiratory Medicine, Unit of Basic Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan.

A 65-year-old Japanese man was referred to our hospital for the further assessment of cough and dyspnea. He had a history of ulcerative colitis for which he was receiving treatment. Chest computed tomography showed a crazy-paving pattern. Read More

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http://dx.doi.org/10.2169/internalmedicine.0555-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6191585PMC
September 2018
5 Reads

Rituximab for auto-immune alveolar proteinosis, a real life cohort study.

Respir Res 2018 04 25;19(1):74. Epub 2018 Apr 25.

Service de Pneumologie A, DHU FIRE, centre de référence constitutif des maladies pulmonaires rares, Hôpital Bichat, APHP, 46 rue Henri Huchard 75877 Paris CEDEX, 18, Paris, France.

Background: Whole lung lavage is the current standard therapy for pulmonary alveolar proteinosis (PAP) that is characterized by the alveolar accumulation of surfactant. Rituximab showed promising results in auto-immune PAP (aPAP) related to anti-GM-CSF antibody.

Methods: We aimed to assess efficacy of rituximab in aPAP in real life and all patients with aPAP in France that received rituximab were retrospectively analyzed. Read More

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http://dx.doi.org/10.1186/s12931-018-0780-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5918901PMC
April 2018
14 Reads