Agammaglobulinemia:
This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discu...
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| Format: | Electronic eBook |
|---|---|
| Language: | English |
| Published: |
Cham
Springer International Publishing
2015
|
| Edition: | 1st ed. 2015 |
| Series: | Rare Diseases of the Immune System
|
| Subjects: | |
| Online Access: | UBR01 Volltext |
| Summary: | This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Bruton's tyrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia. Patients' management in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies are discussed. The book's closing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells |
| Item Description: | Early B Cell Biology -- Agammaglobulinemia: Basic Pathogenesis and Clinical Spectrum (Including X-Linked and Autosomal Recessive Forms) -- Pulmonary Complications in Agammaglobulinemia -- Immunoglobulin Replacement Therapy: Past, Present, Future -- Mutational Spectrum of BTK: A Comprehensive Description -- Novel Therapeutic Options for X-Linked Agammaglobulinemia -- BTK in Non B Cells |
| Physical Description: | 1 Online Ressource (IX, 119 Seiten, 22 Illustrationen, 18 Illustrationen in Farbe) |
| ISBN: | 9783319227146 |
| DOI: | 10.1007/978-3-319-22714-6 |
Staff View
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| 520 | |a This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Bruton's tyrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia. Patients' management in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies are discussed. The book's closing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells | ||
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| dewey-full | 616.079 |
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| discipline | Medizin |
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| doi_str_mv | 10.1007/978-3-319-22714-6 |
| edition | 1st ed. 2015 |
| format | Electronic eBook |
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| id | DE-604.BV046815039 |
| illustrated | Not Illustrated |
| index_date | 2024-07-03T15:00:01Z |
| indexdate | 2024-07-10T08:54:36Z |
| institution | BVB |
| isbn | 9783319227146 |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-032223518 |
| oclc_num | 1190911838 |
| open_access_boolean | |
| owner | DE-355 DE-BY-UBR |
| owner_facet | DE-355 DE-BY-UBR |
| physical | 1 Online Ressource (IX, 119 Seiten, 22 Illustrationen, 18 Illustrationen in Farbe) |
| psigel | ZDB-2-SME ZDB-2-SME_2015 ZDB-2-SME ZDB-2-SME_2015 |
| publishDate | 2015 |
| publishDateSearch | 2015 |
| publishDateSort | 2015 |
| publisher | Springer International Publishing |
| record_format | marc |
| series2 | Rare Diseases of the Immune System |
| spelling | Agammaglobulinemia edited by Alessandro Plebani, Vassilios Lougaris 1st ed. 2015 Cham Springer International Publishing 2015 1 Online Ressource (IX, 119 Seiten, 22 Illustrationen, 18 Illustrationen in Farbe) txt rdacontent c rdamedia cr rdacarrier Rare Diseases of the Immune System Early B Cell Biology -- Agammaglobulinemia: Basic Pathogenesis and Clinical Spectrum (Including X-Linked and Autosomal Recessive Forms) -- Pulmonary Complications in Agammaglobulinemia -- Immunoglobulin Replacement Therapy: Past, Present, Future -- Mutational Spectrum of BTK: A Comprehensive Description -- Novel Therapeutic Options for X-Linked Agammaglobulinemia -- BTK in Non B Cells This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Bruton's tyrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia. Patients' management in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies are discussed. The book's closing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells Immunology Rheumatology Hematology Pediatrics Cell biology Plebani, Alessandro Sonstige oth Lougaris, Vassilios Sonstige oth Erscheint auch als Druck-Ausgabe 9783319227139 Erscheint auch als Druck-Ausgabe 9783319227153 Erscheint auch als Druck-Ausgabe 9783319372495 https://doi.org/10.1007/978-3-319-22714-6 Verlag URL des Erstveröffentlichers Volltext |
| spellingShingle | Agammaglobulinemia Immunology Rheumatology Hematology Pediatrics Cell biology |
| title | Agammaglobulinemia |
| title_auth | Agammaglobulinemia |
| title_exact_search | Agammaglobulinemia |
| title_exact_search_txtP | Agammaglobulinemia |
| title_full | Agammaglobulinemia edited by Alessandro Plebani, Vassilios Lougaris |
| title_fullStr | Agammaglobulinemia edited by Alessandro Plebani, Vassilios Lougaris |
| title_full_unstemmed | Agammaglobulinemia edited by Alessandro Plebani, Vassilios Lougaris |
| title_short | Agammaglobulinemia |
| title_sort | agammaglobulinemia |
| topic | Immunology Rheumatology Hematology Pediatrics Cell biology |
| topic_facet | Immunology Rheumatology Hematology Pediatrics Cell biology |
| url | https://doi.org/10.1007/978-3-319-22714-6 |
| work_keys_str_mv | AT plebanialessandro agammaglobulinemia AT lougarisvassilios agammaglobulinemia |