Autoimmunity in Primary Antibody Deficiencies.

Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)

Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital. Method: All PID cases with a confirmed diagnosis,...

Primary Antibody Deficiencies at Queen Rania Children Hospital in Jordan: Single Center Experience

Background: Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. Objective: This retrospective study aimed at description and analysis of demographic, clinical, im...

B-cell Development and Differentiation

types of primary immune deficiencies in human subjects. Often symptoms do not appear until the latter part of the first year of life, as passively acquired IgG from the mother decreases to below protective levels. As with the T-cell immune deficiencies, the spectrum of antibody deficiencies is broad, ranging from the most severe type of antibody deficiency with totally absent B cells and serum ...

SIAE Rare Variants in Juvenile Idiopathic Arthritis and Primary Antibody Deficiencies

Sialic acid acetylesterase (SIAE) deficiency was suggested to lower the levels of ligands for sialic acid-binding immunoglobulin-like receptors, decreasing the threshold for B-cell activation. In humans, studies of rare heterozygous loss-of-function mutations in SIAE gene in common autoimmune diseases, including juvenile idiopathic arthritis (JIA), yielded inconsistent results. Considering the ...

Possible Role of Autoimmunity in Patients with Premature Ovarian Insuf f iciency