Agammaglobulinemia

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منابع مشابه

BTK (Bruton agammaglobulinemia tyrosine kinase)

The BTK protein is a 77 kDa protein of 659 amino acids. Translation of the BTK transcript starts at the ATG site that is located in exon 2 and ends in exon 19. The BTK protein is composed of an N-terminal Pleckstrin homology (PH) domain followed three protein interacting domains: Tec homology (TH) region, Src homology 3 (SH3) domain and SH2 domain. A tyrosine-kinase catalytic domain is located ...

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[Dermatomyositis-like syndrome in x-linked agammaglobulinemia].

Primary immunodeficiencies (PIDs) encompass more than 250 different pathological conditions. X-linked agammaglobulinemia (XLA) has been occasionally associated with cutaneous and muscular manifestations resembling dermatomyositis, often termed dermatomyositis-like syndrome (DLS). This syndrome has been associated with cutaneous, muscular and central nervous system manifestations, accompanying a...

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Antihelper T cell autoantibody in acquired agammaglobulinemia.

A patient with acquired agammaglobulinemia had an antihelper T cell factor that was identified as an immunoglobulin of the IgG class. The factor specifically bound to the TH2- T cell subset and, in the presence of complement, abolished the helper effect of normal T cells. The antihelper T cell antibody preceded by several years the appearance of suppressor TH2+Ia+ T cells, at which time the cli...

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A clinical and experimental study of agammaglobulinemia.

GAMMAGLOBULINEMlA and extreme hypogam; maglobulinemia occur as concomitants of 'yeral human diseases. Depression of the serum ~roma glob~ concentration may occur be~use of loss of protein from the body, generaled failure of protein fabrication, inordinately . id destruction of serum protein, or because of -isolated deficit in gamma globulin synthesis, n the nephrotic syndrome, for example, hypo...

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X-linked agammaglobulinemia and rheumatoid arthritis

The pathogenic role of B cells in rheumatoid arthritis (RA) has recently gained much interest by the marked clinical responses of anti-CD20 therapy in RA. We describe a patient with X-linked agammaglobulinemia (XLA) who presented with an erosive symmetrical polyarthritis with histological features of RA including formation of a destructive pannus. Furthermore, the patient also developed subcuta...

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ژورنال

عنوان ژورنال: Japanese Journal of Clinical Immunology

سال: 2002

ISSN: 0911-4300,1349-7413

DOI: 10.2177/jsci.25.126