Titlebook: Agammaglobulinemia; Alessandro Plebani,Vassilios Lougaris Book 2015 Springer International Publishing Switzerland 2015 Autosomal recessive

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,Bruton’s Tyrosine Kinase (BTK) Beyond B Lymphocytes: A Protein Kinase with Relevance in Innate Immulls of human and murine origin and have identified its role in controlling different aspects of the innate immune response and in diverse pathologies. This chapter summarizes these findings and discusses the possible implications of BTK deficiency in non-B cells involved in the phenotype of human and murine primary immunodeficiencies.

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Trevor MacLean,Robert Borrelli,Michael Haneyoduction, and impaired airway clearance leads to structural airway damage, in particular to bronchiectasis. This chapter discusses the diagnostic and therapeutic approach to the patient with agammaglobulinemia with lung disease. The discussion refers to the available evidence with XLA patients and t

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J. Peter Brady,Sergey Bratus,Sean Smithg serum proteins into six fractions, with one (fraction II) containing gammaglobulin, paved the way for the generation of safe and effective immunoglobulin G (IgG) preparations that can be given intravenously or subcutaneously in an infusion center or at home by an experienced nurse or by self-infus

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Quantifying the Costs of Data Breachess, which are only partially effective and not curative. Therefore, novel treatment options including bone marrow transplantation and gene therapy are being developed. For gene therapy, autologous stem cells in which lentiviruses are used to correct the BTK gene deficiency are in preclinical stage. A

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Trevor MacLean,Robert Borrelli,Michael Haneyfor early B cell development in the bone marrow and for B cell activation and differentiation in peripheral lymphoid organs. In addition to its role in B cell development and function, BTK is also expressed in myeloid cell populations. Several groups have investigated the function of BTK in non-B ce

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Agammaglobulinemia978-3-319-22714-6Series ISSN 2282-6505 Series E-ISSN 2283-6403