نتایج جستجو برای: primary immunodeficiency
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Background: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disease, characterized by hypogammaglobulinemia and heterogeneous clinical manifestations. This study was performed to evaluate the clinical and immunological features of pediatric patients with CVID. Methods: We reviewed the records of 69 children diagnosed under age of 16 years with CVID (35 males and 34 ...
co-infection of tuberculosis and leishmaniasis has been reported previously in patients with altered immune response due to primary or secondary immunodeficiency. these intracellular pathogens have a unique interaction in the host immune response and their development depends on impaired cell-mediated immunity. a case of chronic non-healing cutaneous ulcer with chronic fistulating soft-tissue m...
a new il-2rg gene mutation in an x-linked scid identified through trec/krec screening: a case report
severe combined immunodeficiency (scid) represents a rare group of primary immunodeficiency disorders (pids), with known or unknown genetic alterations. here, we report a new interleukin 2 receptor, gamma chain (il-2rg) mutation in an iranian scid newborn.the patient was a 6-day old boy with a family history of pid. the child was screened using a molecular-based analysis for the assessment of t...
Primary immunodeficiencies (PIDs) are genetic disorders that predispose to frequent and severe infections, autoimmunity and cancer. The expanded life span of such patients increases the overall risk for developing cancer, which is now estimated at 4-25% . The type of malignancy depends on the primary immunodeficiency, the age of the patient and possible viral infection, suggesting that differen...
Introduction: In tuberculosis (TB) endemic regions BCG vaccine is administered at birth in an effort to protect against neonatal tuberculous meningitis. However, this live vaccine facilitates overwhelming systemic infections by otherwise innocuous organisms in infants with cellular primary immunodeficiencies . Case Report: Our case is a seven month old infant who developed abscess at BCG vaccin...
Artemis, DNA ligase IV, DNA protein kinase catalytic subunit, and Cernunnos/XLF genes in nonhomologous end joining pathways of DNA repair mechanisms have been identified as responsible for radiosensitive SCID. Here, we present a 3-year-old girl patient with severe growth retardation, bird-like face, recurrent perianal abscess, pancytopenia, and polydactyly. Firstly, she was thought as Fanconi a...
One in 2,000 children younger than 18 years is thought to have a primary immunodeficiency disease. Antibody, combined B-cell and T-cell, phagocytic, and complement disorders are the most common types. Children with these diseases tend to have bacterial or fungal infections with unusual organisms, or unusually severe and recurrent infections with common organisms. A family history of primary imm...
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