Progression of Selective IgA Deficiency to Common Variable Immunodeficiency

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Progression of selective IgA deficiency to common variable immunodeficiency.

Selective IgA deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Although it is often asymptomatic, selected patients show an increased frequency of infections, allergies and autoimmune manifestations. Common variable immunodeficiency (CVID) is a primary antibody deficiency disease that shares many clinical features with IgAD. A common genetic basis for IgAD and CVID h...

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Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency.

FIRST REPORT: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as IgA deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. Several immunologic evaluations were made between the ages of 4 months and 8 years. At 8 years and 9 months, the diagnosis of IgA deficiency was confirmed, and asso...

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Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency.

Interleukin-21 (IL-21) is an important promoter for differentiation of human B cells into immunoglobulin (Ig)-secreting cells. The objective of this study was to evaluate an IL-21-based approach to induce immunoglobulin production in B cells from patients with common variable immunodeficiency (CVID) or selective IgA deficiency (IgAD). We show that a combination of IL-21, IL-4, and anti-CD40 sti...

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Terminally differentiated memory T cells are increased in patients with common variable immunodeficiency and selective IgA deficiency

Introduction Previous studies showed that several lymphocyte abnormalities seen in the most frequent symptomatic immunoglobulin deficiency, common variable immunodeficiency (CVID), were also observed in a genetically related asymptomatic disorder - selective IgA deficiency (IgAD). In this study we searched for abnormalities in the differentiation stages of T cells as well as for similarities of...

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Role of apoptosis in common variable immunodeficiency and selective immunoglobulin A deficiency.

Common variable immunodeficiency (CVID) and selective IgA deficiency (SIgAD) are the most common primary immunodeficiencies in human. Both diseases share clinical manifestation and molecular defects. Increased apoptosis may be one of the mechanisms involved in the pathogenesis of CVID and SIgAD. Elevated apoptosis in this disorder leads to defective long-term survival of B-cells, reduced antibo...

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ژورنال

عنوان ژورنال: International Archives of Allergy and Immunology

سال: 2008

ISSN: 1423-0097,1018-2438

DOI: 10.1159/000135694