Comprehensive review of autoantibodies in patients with hyper-IgM syndrome
نویسندگان
چکیده
منابع مشابه
Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis
Background: Defects in B cell class switch recombination (CSR) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper IgM (HIgM) syndrome. Due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients. Objective: To shed some light on the morbidity and mortality regar...
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hyper igm syndrome (him sx) is a rare congenital primary immunodeficiency that affects males more than females (70%, x-iinked recessive), but there are reports of autosomal recessive and autosomal dominant inheritances. in this study, we review medical histories of 2 affected girls and one affected boy. our 3 cases fulfill clinical and laboratory criteria of this syndrome. their clinical signs ...
متن کاملDefective self-reactive antibody repertoire of serum IgM in patients with hyper-IgM syndrome.
We have analyzed the self-reactive repertoires of IgM and IgG Abs in the serum of 19 patients with hyper-IgM syndrome (HIM) by means of a quantitative immunoblotting technique that allows for a quantitative comparison of Ab repertoires in health and disease by multiparametric statistical analysis. Normal tissue extracts of liver, lung, stomach, and kidney were used as sources of self Ags. Extra...
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The clinical and immunological aspects of 16 children with the syndrome of hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome) and their responses to treatment are reviewed. Increased concentrations of IgM, neutropenia, and recurrent infections could usually be controlled by antimicrobial and intravenous immunoglobulin treatment. Together with the bacterial i...
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ژورنال
عنوان ژورنال: Cellular & Molecular Immunology
سال: 2018
ISSN: 1672-7681,2042-0226
DOI: 10.1038/cmi.2017.140