2,001 results match your criteria Pulmonary Alveolar Proteinosis


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

J Immunol 2019 Jan 16. 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
January 2019

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
1 Read

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
2 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
5 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
1 Read

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

Lancet Respir Med 2018 Nov;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

Blood testing in the diagnosis of pulmonary alveolar proteinosis.

Lancet Respir Med 2018 Nov;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
1 Read

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

Orphanet J Rare Dis 2018 Oct 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
3 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
4 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
1 Read

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
11 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
17 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
20 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
1 Read

Prevalence and healthcare burden of pulmonary alveolar proteinosis.

Orphanet J Rare Dis 2018 Jul 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
14 Reads

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

Lancet Respir Med 2018 Sep 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
2 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
10 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
2 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
May 2017
4 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
7 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
2 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
9 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
13 Reads

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

MAbs 2018 Oct 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
9 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
2 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
4 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
3 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
6 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
6 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
3 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
9 Reads

Adenosine deaminase deficiency: a review.

Orphanet J Rare Dis 2018 Apr 24;13(1):65. Epub 2018 Apr 24.

Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.

Adenosine deaminase (ADA) deficiency leads to an accumulation of toxic purine degradation by-products, most potently affecting lymphocytes, leading to adenosine deaminase-deficient severe combined immunodeficiency. Whilst most notable affects are on lymphocytes, other manifestations include skeletal abnormalities, neurodevelopmental affects and pulmonary manifestations associated with pulmonary-alveolar proteinosis. Affected patients present in early infancy, usually with persistent infection, or with pulmonary insufficiency. Read More

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http://dx.doi.org/10.1186/s13023-018-0807-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5916829PMC
April 2018
4 Reads

The Use of High-Frequency Percussive Ventilation for Whole-Lung Lavage: A Case Report.

A A Pract 2018 Oct;11(8):205-207

Cardiothoracic Surgery, New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York.

Whole-lung lavage (WLL) remains the gold standard in the treatment of pulmonary alveolar proteinosis. However, anesthetic management during WLL can be challenging because of the risk of intraoperative hypoxemia and various cardiorespiratory complications of 1-lung ventilation. Here, we describe a novel strategy involving the application of high-frequency percussive ventilation using a volumetric diffusive respirator (VDR-4) during WLL in a 47-year-old woman with pulmonary alveolar proteinosis. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000778DOI Listing
October 2018
2 Reads

Manmade Macrophage Offers a New Therapy for Pulmonary Alveolar Proteinosis.

Am J Respir Crit Care Med 2018 Aug;198(3):297-298

3 Ansary Stem Cell Institute Weill Cornell Medicine New York, New York.

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http://dx.doi.org/10.1164/rccm.201803-0478EDDOI Listing
August 2018
3 Reads

Whole-lung lavage in a patient with pulmonary alveolar proteinosis.

Ann Card Anaesth 2018 Apr-Jun;21(2):215-217

Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, MN, USA.

Pulmonary alveolar proteinosis (PAP) is a rare syndrome in which phospholipoproteinaceous matter accumulates in the alveoli leading to compromised gas exchange. Whole-lung lavage is considered the gold standard for severe autoimmune PAP and offers favorable long-term outcomes. In this case report, we describe the perioperative management and procedural specifics of a patient undergoing WLL for PAP in which an anesthesiologist serves as the proceduralist and a separate anesthesiologist provides anesthesia care for the patient. Read More

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http://dx.doi.org/10.4103/aca.ACA_184_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5914230PMC
November 2018
5 Reads

Pulmonary Transplantation of Human Induced Pluripotent Stem Cell-derived Macrophages Ameliorates Pulmonary Alveolar Proteinosis.

Am J Respir Crit Care Med 2018 Aug;198(3):350-360

1 Department of Pediatric Pneumology, Allergology and Neonatology.

Rationale: Although the transplantation of induced pluripotent stem cell (iPSC)-derived cells harbors enormous potential for the treatment of pulmonary diseases, in vivo data demonstrating clear therapeutic benefits of human iPSC-derived cells in lung disease models are missing.

Objectives: We have tested the therapeutic potential of iPSC-derived macrophages in a humanized disease model of hereditary pulmonary alveolar proteinosis (PAP). Hereditary PAP is caused by a genetic defect of the GM-CSF (granulocyte-macrophage colony-stimulating factor) receptor, which leads to disturbed macrophage differentiation and protein/surfactant degradation in the lungs, subsequently resulting in severe respiratory insufficiency. Read More

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https://www.atsjournals.org/doi/10.1164/rccm.201708-1562OC
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http://dx.doi.org/10.1164/rccm.201708-1562OCDOI Listing
August 2018
16 Reads

Supramolecular Assembly of Human Pulmonary Surfactant Protein SP-D.

J Mol Biol 2018 May 4;430(10):1495-1509. Epub 2018 Apr 4.

Department of Biochemistry, Faculty of Biology, Complutense University, 28040 Madrid, Spain; Research Institut "Hospital 2 de Octubre (imas12)", 28040 Madrid, Spain. Electronic address:

Pulmonary surfactant protein D (SP-D) is a glycoprotein from the collectin family that is a component of the lung surfactant system. It exhibits host defense and immune regulatory functions in addition to contributing to the homeostasis of the surfactant pool within the alveolar airspaces. It is known that the SP-D monomer forms trimers, which further associate into higher-order oligomers. Read More

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http://dx.doi.org/10.1016/j.jmb.2018.03.027DOI Listing
May 2018
3 Reads

Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse.

Case Rep Pulmonol 2018 23;2018:5202173. Epub 2018 Jan 23.

Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada.

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoimmune, and secondary. We report a case of secondary PAP in a 47-year-old man, whose risk factors include occupational exposure to inhaled toxins, especially aluminum dust, the use of anabolic steroids, and alcohol abuse, which in mice leads to alveolar macrophage dysfunction through a zinc-dependent mechanism that inhibits granulocyte macrophage-colony stimulating factor (GM-CSF) receptor signalling. Read More

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http://dx.doi.org/10.1155/2018/5202173DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5828087PMC
January 2018
3 Reads

Pulmonary alveolar proteinosis following cryptococcal meningitis: a possible cause?

BMJ Case Rep 2018 Mar 28;2018. Epub 2018 Mar 28.

Department of Pneumology, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Lyon, France.

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterised by the presence of granulocyte macrophage colony-stimulating factor (GM-CSF) autoantibodies. A man with no history of infection developed cryptococcal meningitis and a right parahilar cryptococcal mass. Antifungal treatment led to infection control, although there was presence of neurological sequelae. Read More

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http://dx.doi.org/10.1136/bcr-2017-222940DOI Listing
March 2018
5 Reads

Evolving management and patient choice in pulmonary alveolar proteinosis.

Lancet Respir Med 2018 07 23;6(7):e35. Epub 2018 Mar 23.

Department of Respiratory Medicine, The Alfred Hospital, Melbourne, VIC 3004, Australia. Electronic address:

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http://dx.doi.org/10.1016/S2213-2600(18)30107-3DOI Listing
July 2018
3 Reads

Genetic causes and clinical management of pediatric interstitial lung diseases.

Curr Opin Pulm Med 2018 05;24(3):253-259

Service de pneumologie pédiatrique, Hôpital Armand Trousseau, Assistance Publique Hôpitaux de Paris (AP-HP), Centre national de référence des maladies respiratoires rares RespiRare.

Purpose Of Review: Interstitial lung disease (ILD) in children (chILD) is an umbrella term for 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 implicates genetic contributors. The purpose of this review is to provide updated information on the molecular defects associated with the development of chILD. Read More

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http://dx.doi.org/10.1097/MCP.0000000000000471DOI Listing
May 2018
5 Reads

Pleural effusion as presenting feature of pulmonary alveolar proteinosis: A rare occurrence.

Clin Respir J 2018 Jul 26;12(7):2300-2302. Epub 2018 Mar 26.

Pathology & Lab Medicine, AIIMS, Bhopal, India.

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http://dx.doi.org/10.1111/crj.12788DOI Listing
July 2018
8 Reads