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  1. Book: Agammaglobulinemia

    Plebani, Alessandro / Lougaris, Vassilios

    (Rare diseases of the immune system)

    2015  

    Author's details Alessandro Plebani ; Vassilios Lougaris ed
    Series title Rare diseases of the immune system
    Keywords Autosomal recessive agammaglobulinemia ; Bruton tyrosine kinase ; Pre-B cell receptor ; B cells ; Bruton’s agammaglobulinemia ; Gammaglobulin replacement therapy
    Language English
    Size IX, 119 S. : Ill., graph. Darst.
    Publisher Springer
    Publishing place Cham u.a.
    Publishing country Switzerland
    Document type Book
    HBZ-ID HT018801054
    ISBN 978-3-319-22713-9 ; 978-3-319-22714-6 ; 3-319-22713-0 ; 3-319-22714-9
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2015 A 3731 available for loan (reading room) Lesesaal EG

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  2. Article ; Online: Microvascular and Macrovascular Endothelial Cell Isolation and Purification from Lung-Derived Samples.

    Plebani, Roberto / D'Alessandro, Alessandra / Lanuti, Paola / Simeone, Pasquale / Cinalli, Massimiliano / Righi, Ilaria / Palleschi, Alessandro / Mucci, Matteo / Marchisio, Marco / Cappabianca, Francesco / Camera, Marina / Mucilli, Felice / Romano, Mario

    Journal of visualized experiments : JoVE

    2023  , Issue 192

    Abstract: The availability of cells isolated from healthy and diseased tissues and organs represents a key element for personalized medicine approaches. Although biobanks can provide a wide collection of primary and immortalized cells for biomedical research, ... ...

    Abstract The availability of cells isolated from healthy and diseased tissues and organs represents a key element for personalized medicine approaches. Although biobanks can provide a wide collection of primary and immortalized cells for biomedical research, these do not cover all experimental needs, particularly those related to specific diseases or genotypes. Vascular endothelial cells (ECs) are key components of the immune inflammatory reaction and, thus, play a central role in the pathogenesis of a variety of disorders. Notably, ECs from different sites display different biochemical and functional properties, making the availability of specific EC types (i.e., macrovascular, microvascular, arterial, and venous) essential for designing reliable experiments. Here, simple procedures to obtain high-yield, virtually pure human macrovascular and microvascular endothelial cells from the pulmonary artery and lung parenchyma are illustrated in detail. This methodology can be easily reproduced at a relatively low cost by any laboratory to achieve independence from commercial sources and obtain EC phenotypes/genotypes that are not yet available.
    MeSH term(s) Humans ; Endothelial Cells ; Endothelium, Vascular ; Lung ; Cell Line ; Cell Separation ; Cells, Cultured
    Language English
    Publishing date 2023-02-03
    Publishing country United States
    Document type Journal Article ; Video-Audio Media ; Research Support, Non-U.S. Gov't
    ZDB-ID 2259946-0
    ISSN 1940-087X ; 1940-087X
    ISSN (online) 1940-087X
    ISSN 1940-087X
    DOI 10.3791/64885
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: In adult X-CGD patients, regulatory T cells are expanded while activated T cells display a NOX2-independent ROS increase.

    Cammarata, Ilenia / Pinna, Valeria / Pacella, Ilenia / Rotella, Ivano / Soresina, Annarosa / Badolato, Raffaele / Plebani, Alessandro / Pignata, Claudio / Cirillo, Emilia / Zicari, Anna Maria / Violi, Francesco / Carnevale, Roberto / Loffredo, Lorenzo / Piconese, Silvia

    Immunology letters

    2024  Volume 266, Page(s) 106839

    Abstract: The X-linked chronic granulomatous disease (X-CGD), a rare genetic disease characterised by recurrent infections, is caused by mutations of NOX2. Significant proportions of X-CGD patients display signs of immune dysregulation. Regulatory T cells (Tregs) ... ...

    Abstract The X-linked chronic granulomatous disease (X-CGD), a rare genetic disease characterised by recurrent infections, is caused by mutations of NOX2. Significant proportions of X-CGD patients display signs of immune dysregulation. Regulatory T cells (Tregs) are CD4
    MeSH term(s) Adult ; Humans ; Granulomatous Disease, Chronic/genetics ; Granulomatous Disease, Chronic/metabolism ; T-Lymphocytes, Regulatory ; NADPH Oxidases/genetics ; NADPH Oxidases/metabolism ; Reactive Oxygen Species/metabolism ; Mutation
    Chemical Substances NADPH Oxidases (EC 1.6.3.-) ; Reactive Oxygen Species
    Language English
    Publishing date 2024-02-01
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 445150-8
    ISSN 1879-0542 ; 0165-2478
    ISSN (online) 1879-0542
    ISSN 0165-2478
    DOI 10.1016/j.imlet.2024.106839
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1512: Show issues Location:
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  4. Article ; Online: Diagnostic approach of hypogammaglobulinemia in infancy.

    Plebani, Alessandro / Palumbo, Laura / Dotta, Laura / Lougaris, Vassilios

    Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology

    2020  Volume 31 Suppl 24, Page(s) 11–12

    Abstract: Primary B-cell immunodeficiency is the most frequent immune defect in infancy. Selective absence of serum and secretory immunoglobulin IgA is the most common, with rates ranging from 1/333 persons to 1/16 000, among different races. By contrast, it has ... ...

    Abstract Primary B-cell immunodeficiency is the most frequent immune defect in infancy. Selective absence of serum and secretory immunoglobulin IgA is the most common, with rates ranging from 1/333 persons to 1/16 000, among different races. By contrast, it has been estimated that hypo/agammaglobulinemia occurs with a frequency of 1/50 000 persons. Patients with antibody deficiency are usually recognized because they have recurrent infections with encapsulated bacteria or a history of failure to respond adequately to antibiotic treatment. However, some individuals, mainly those affected by IgA deficiency (SIgAD) or transient hypogammaglobulinemia of infancy , may have few or no infections.
    MeSH term(s) Agammaglobulinemia/diagnosis ; Agammaglobulinemia/genetics ; B-Lymphocytes/immunology ; CD40 Ligand/genetics ; Humans ; Hyper-IgM Immunodeficiency Syndrome, Type 1/diagnosis ; Hyper-IgM Immunodeficiency Syndrome, Type 1/genetics ; IgA Deficiency/diagnosis ; IgA Deficiency/genetics ; Immunoglobulin A/genetics ; Infant ; Infant, Newborn ; Infections/diagnosis ; Infections/genetics ; Lymphocyte Activation ; Recurrence
    Chemical Substances Immunoglobulin A ; CD40 Ligand (147205-72-9)
    Language English
    Publishing date 2020-02-11
    Publishing country England
    Document type Journal Article
    ZDB-ID 1057059-7
    ISSN 1399-3038 ; 0905-6157 ; 0906-5784
    ISSN (online) 1399-3038
    ISSN 0905-6157 ; 0906-5784
    DOI 10.1111/pai.13166
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 3096: Show issues Location:
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  5. Book: Agammaglobulinemia

    Plebani, Alessandro / Lougaris, Vassilios

    (Rare diseases of the immune system,)

    2015  

    Author's details Alessandro Plebani, Vassilios Lougaris, editors
    Series title Rare diseases of the immune system,
    MeSH term(s) Agammaglobulinemia
    Language English
    Size ix, 119 pages :, illustrations.
    Document type Book
    ISBN 9783319227139 ; 9783319227146 ; 3319227130 ; 3319227149
    Database Catalogue of the US National Library of Medicine (NLM)

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  6. Article ; Online: RAC2 and primary human immune deficiencies.

    Lougaris, Vassilios / Baronio, Manuela / Gazzurelli, Luisa / Benvenuto, Alessio / Plebani, Alessandro

    Journal of leukocyte biology

    2020  Volume 108, Issue 2, Page(s) 687–696

    Abstract: RAC2 is a GTPase that is exclusively expressed in hematopoietic cells. Animal models have suggested important roles for RAC2 in the biology of different cell types, such as neutrophils and lymphocytes. Primary immunodeficiencies represent "experimentum ... ...

    Abstract RAC2 is a GTPase that is exclusively expressed in hematopoietic cells. Animal models have suggested important roles for RAC2 in the biology of different cell types, such as neutrophils and lymphocytes. Primary immunodeficiencies represent "experimentum naturae" and offer priceless insight on the function of the human immune system. Mutations in RAC2 have been identified in a small number of patients giving rise to different forms of primary immunodeficiencies ranging from granulocyte defects caused by dominant negative mutations to combined immunodeficiency due to dominant activating mutations. This review will focus on the clinical and immunologic phenotype of patients with germline mutations in RAC2.
    MeSH term(s) Animals ; Biomarkers ; Disease Susceptibility/immunology ; Genetic Predisposition to Disease ; Germ-Line Mutation ; Humans ; Multigene Family ; Mutation ; Primary Immunodeficiency Diseases/etiology ; Primary Immunodeficiency Diseases/metabolism ; rac GTP-Binding Proteins/genetics ; rac GTP-Binding Proteins/metabolism ; RAC2 GTP-Binding Protein
    Chemical Substances Biomarkers ; rac GTP-Binding Proteins (EC 3.6.5.2)
    Language English
    Publishing date 2020-06-15
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 605722-6
    ISSN 1938-3673 ; 0741-5400
    ISSN (online) 1938-3673
    ISSN 0741-5400
    DOI 10.1002/JLB.5MR0520-194RR
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 603: Show issues Location:
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    ab Jg. 2022: Lesesaal (EG)

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  7. Article ; Online: Lymphocyte alterations in patients with Common Variable Immunodeficiency (CVID) and autoimmune manifestations.

    Rossi, Stefano / Baronio, Manuela / Gazzurelli, Luisa / Tessarin, Giulio / Baresi, Giulia / Chiarini, Marco / Moratto, Daniele / Badolato, Raffaele / Plebani, Alessandro / Lougaris, Vassilios

    Clinical immunology (Orlando, Fla.)

    2022  Volume 241, Page(s) 109077

    Abstract: Introduction: Autoimmunity is a common feature in CVID patients. To date the mechanisms leading to the development of such complications are not fully elucidated.: Materials and methods: Data from 122 CVID patients subdivided in three groups based on ...

    Abstract Introduction: Autoimmunity is a common feature in CVID patients. To date the mechanisms leading to the development of such complications are not fully elucidated.
    Materials and methods: Data from 122 CVID patients subdivided in three groups based on the absence of autoimmunity (n-AI) or the presence of hematologic autoimmune phenomena (Cy-AI) or non-hematologic autoimmune phenomena (n-Cy-AI) were evaluated.
    Results: We identified a total of 128 autoimmune manifestations in 55/122 patients (45.1%). 30/122 (24.6%) patients presented hematologic autoimmune phenomena while 29/122 (23.8%) presented gastrointestinal autoimmune involvement. Immune thrombocytopenia was the most common manifestation (27/122; 22.1%), followed by autoimmune hemolytic anemia (18/122; 14.8%) and autoimmune enteropathy (17/122; 13.9%). Cy-AI patients displayed higher CD4
    Conclusions: CVID patients developing autoimmune cytopenias display characteristic immune phenotypic features.
    MeSH term(s) Autoimmunity ; CD4-Positive T-Lymphocytes ; Common Variable Immunodeficiency ; Humans ; Immunophenotyping ; Purpura, Thrombocytopenic, Idiopathic
    Language English
    Publishing date 2022-07-14
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1459903-x
    ISSN 1521-7035 ; 1521-6616
    ISSN (online) 1521-7035
    ISSN 1521-6616
    DOI 10.1016/j.clim.2022.109077
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Response to the Letter to the Editor Regarding "Kinetics of Radiological Response of Thoracic Invasive Fungal Disease in Chronic Granulomatous Disease".

    Bondioni, Maria P / Lougaris, Vassilios / Plebani, Alessandro

    Journal of clinical immunology

    2017  Volume 37, Issue 8, Page(s) 744–745

    MeSH term(s) Adolescent ; Antifungal Agents/therapeutic use ; Granulomatous Disease, Chronic/diagnosis ; Granulomatous Disease, Chronic/drug therapy ; Humans ; Invasive Fungal Infections/diagnosis ; Invasive Fungal Infections/drug therapy ; Lung/diagnostic imaging ; Lung/pathology ; Tomography, X-Ray Computed/methods ; Treatment Outcome
    Chemical Substances Antifungal Agents
    Language English
    Publishing date 2017-10-08
    Publishing country Netherlands
    Document type Letter
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-017-0450-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: When a Nontuberculous Mycobacterial Infection Reveals an Error of Immunity: A Single Center's Experience.

    Morreale, Alessia / Dotta, Laura / Vairo, Donatella / Bazzana, Tullia / Lougaris, Vassilios / Soresina, Annarosa / Plebani, Alessandro / Giliani, Silvia Clara / Porta, Fulvio / Matteelli, Alberto / Redaelli De Zinis, Luca Oscar / Badolato, Raffaele

    The Pediatric infectious disease journal

    2022  Volume 41, Issue 5, Page(s) 427–429

    Abstract: We present an algorithm that may be applied in case of a diagnosis of pediatric nontuberculous mycobacterial disease to identify the patients who may require an immunologic assessment to discover a possible underlying immune system defect predisposing to ...

    Abstract We present an algorithm that may be applied in case of a diagnosis of pediatric nontuberculous mycobacterial disease to identify the patients who may require an immunologic assessment to discover a possible underlying immune system defect predisposing to their nontuberculous mycobacterial infections.
    MeSH term(s) Child ; Humans ; Mycobacterium Infections, Nontuberculous/diagnosis ; Mycobacterium Infections, Nontuberculous/microbiology ; Nontuberculous Mycobacteria
    Language English
    Publishing date 2022-01-27
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 392481-6
    ISSN 1532-0987 ; 0891-3668
    ISSN (online) 1532-0987
    ISSN 0891-3668
    DOI 10.1097/INF.0000000000003461
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Mycoplasma infection may complicate the clinical course of SARS-Co-V-2 associated Kawasaki-like disease in children.

    Plebani, Alessandro / Meini, Antonella / Cattalini, Marco / Lougaris, Vassilios / Bugatti, Antonella / Caccuri, Francesca / Caruso, Arnaldo

    Clinical immunology (Orlando, Fla.)

    2020  Volume 221, Page(s) 108613

    MeSH term(s) Adolescent ; Antibodies, Bacterial/blood ; Blotting, Western ; COVID-19/complications ; COVID-19/diagnosis ; COVID-19/pathology ; COVID-19/virology ; COVID-19 Testing ; Child ; Child, Preschool ; Coinfection ; Conjunctivitis/diagnosis ; Conjunctivitis/physiopathology ; Edema/diagnosis ; Edema/physiopathology ; Exanthema/diagnosis ; Exanthema/physiopathology ; Female ; Fever/diagnosis ; Fever/physiopathology ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome/complications ; Mucocutaneous Lymph Node Syndrome/diagnosis ; Mucocutaneous Lymph Node Syndrome/pathology ; Mucocutaneous Lymph Node Syndrome/virology ; Mycoplasma pneumoniae/growth & development ; Mycoplasma pneumoniae/pathogenicity ; Pneumonia, Mycoplasma/complications ; Pneumonia, Mycoplasma/diagnosis ; Pneumonia, Mycoplasma/microbiology ; Pneumonia, Mycoplasma/pathology ; SARS-CoV-2/growth & development ; SARS-CoV-2/pathogenicity ; Severe Acute Respiratory Syndrome/complications ; Severe Acute Respiratory Syndrome/diagnosis ; Severe Acute Respiratory Syndrome/pathology ; Severe Acute Respiratory Syndrome/virology ; Stomatitis/diagnosis ; Stomatitis/physiopathology
    Chemical Substances Antibodies, Bacterial
    Keywords covid19
    Language English
    Publishing date 2020-10-16
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1459903-x
    ISSN 1521-7035 ; 1521-6616
    ISSN (online) 1521-7035
    ISSN 1521-6616
    DOI 10.1016/j.clim.2020.108613
    Database MEDical Literature Analysis and Retrieval System OnLINE

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