نتایج جستجو برای: t cell immunodeficiency primary
تعداد نتایج: 2753082 فیلتر نتایج به سال:
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,...
HIE is a primary immunodeficiency characterized by recurrent staphylococcal abscesses and markedly elevated serum IgE concentrations. These abcesses involve the skin, lungs, joints, and other sites, and there is a unique tendency to form pneumatoceles following staphylococcal pneumonias. Although, there is as yet no clearly defined immunologic cause for this rare syndrome, but some form of T-Ce...
The underlying cause of the enigmatic coexistence of immunodeficiency and autoimmune disorders in patients with primary immunodeficiency such as Omenn syndrome is largely due to the inefficient negative selection within thymus where T cells develop. Recent advances in molecular biology and animal models answered one of the key questions on the relationship between the partially impaired T cell ...
Background: Hyper-Immunoglobulin M (IgM) syndrome (HIGM) is a rare primary immunodeficiency in which defective B-cell isotype switching results in a phenotype characterized by elevated or normal serum IgM levels and low levels of other Ig classes, leading to an increased susceptibility to infection, neutropenia, autoimmune disorders, and malignancies. In this disease, a mutation occurs in the C...
Patients with hypogammaglobulinemia who do not fulfill all the classical diagnostic criteria for common variable immunodeficiency (reduction of two immunoglobulin isotypes and a reduced response to vaccination) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients with idiopathic primary hypogammaglobulin...
primary immunodeficiency diseases (pids) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. the aim of this study was to assess the knowledge of iranian general practitioners and pediatricians about pids. a questionnaire consisting 52 closed questions on clinical symptoms, laboratory data,associated syndromes and management of pids patients wa...
primary immunodeficiency disorders are a heterogeneous group of genetic disorders, with different modes of inheritance, consisting of more than 100 different types. we constructed the dna banking of primary immunodeficiency disorders for the first time in iran. the dna of 31 immunodeficient patients and their families (total of 92 samples) were collected, as the first step for construction of d...
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