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Agammaglobulinemia[electronic resour...

Lougaris, Vassilios.

Agammaglobulinemia[electronic resource] /

レコード種別:	言語・文字資料 (印刷物) : 単行資料
[NT 15000414] null:	616.979
タイトル / 著者:	Agammaglobulinemia/ edited by Alessandro Plebani, Vassilios Lougaris.
その他の著者:	Plebani, Alessandro.
出版された:	Cham : : Springer International Publishing :, 2015.
記述:	ix, 119 p. : : ill., digital ;; 24 cm.
含まれています:	Springer eBooks
主題:	Agammaglobulinemia.
主題:	Agammaglobulinemia - Treatment.
主題:	Biomedicine.
主題:	Immunology.
主題:	Rheumatology.
主題:	Hematology.
主題:	Pediatrics.
主題:	Cell Biology.
国際標準図書番号 (ISBN) :	9783319227146
国際標準図書番号 (ISBN) :	9783319227139
[NT 15000228] null:	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.
[NT 15000229] null:	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.
電子資源:	http://dx.doi.org/10.1007/978-3-319-22714-6

Agammaglobulinemia[electronic resource] /
Agammaglobulinemia[electronic resource] /edited by Alessandro Plebani, Vassilios Lougaris. - Cham :Springer International Publishing :2015. - ix, 119 p. :ill., digital ;24 cm. - Rare diseases of the immune system,42282-6505 ;. - Rare diseases of the immune system ;4..

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.

ISBN: 9783319227146

Standard No.: 10.1007/978-3-319-22714-6doiSubjects--Topical Terms:

635935
Agammaglobulinemia.

LC Class. No.: RC606

Dewey Class. No.: 616.979

Agammaglobulinemia[electronic resource] /
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245 0 0 $a Agammaglobulinemia $h [electronic resource] / $c edited by Alessandro Plebani, Vassilios Lougaris.
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300 $a ix, 119 p. : $b ill., digital ; $c 24 cm.
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505 0 $a 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.
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.
650 0 $a Agammaglobulinemia. $3 635935
650 0 $a Agammaglobulinemia $x Treatment. $3 635936
650 1 4 $a Biomedicine. $3 463454
650 2 4 $a Immunology. $3 402223
650 2 4 $a Rheumatology. $3 429601
650 2 4 $a Hematology. $3 381913
650 2 4 $a Pediatrics. $3 381606
650 2 4 $a Cell Biology. $3 463594
700 1 $a Plebani, Alessandro. $3 635932
700 1 $a Lougaris, Vassilios. $3 635933
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830 0 $a Rare diseases of the immune system ; $v 4. $3 635934
856 4 0 $u http://dx.doi.org/10.1007/978-3-319-22714-6
950 $a Medicine (Springer-11650)

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http://dx.doi.org/10.1007/978-3-319-22714-6

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