HYPER - IgM SYNDROME WITH LYMPHOID HYPERPLASIA 77
نویسندگان
چکیده
منابع مشابه
Hyper-IgM syndrome: a case report.
Hyperimmunoglobulin M syndrome is a rare primary immunodeficiency disorder. We report a case of a 6-month-old boy who suffered from developmental delays, frequent respiratory tract infection, and unusual fungal and bacterial infection. X-linked hyperimmunoglobulin M syndrome was ultimately diagnosed with decreasing immunoglobulin-G, A, and E (immunoglobulin G = 51.3 mg/dL, immunoglobulin A = 8....
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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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INDIAN PEDIATRICS 952 VOLUME 42__SEPTEMBER 17, 2005 Hyper IgM syndrome (HIGM) is a rare primary immunodeficiency characterized by low levels of serum IgA and IgG and a normal or increased levels of IgM due to a block in the B cell immunoglobulin switch from IgM to IgG(1). The estimated incidence is approximately 1 in 10,30,000 live births(2). Two types are described X linked hyper IgM (XHIM) an...
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Hyper-IgM syndrome (HIM) is a rare disorder resulting from mutation in the CD40 ligand (CD40L) gene. This defect is associated with normal or elevated serum level of IgM and with low to undetectable levels of serum IgG, IgA, IgE. This case of HIM with CD40L deficiency was proven by flow cytometry but initially presented as interstitial pneumonia. Pneumocystis carinii pneumonia was highly sugges...
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Eosinophilia in the absence of allergies, asthma, drug reactions, parasitic infections and connective tissue diseases can be eosinophilic clonal disorders, lymphoma or myeloproliferative disorders. Hypereosinophilia with the persistence of eosinophils ≥1500 /mm³ in blood or more than 20% of eosinophils in the bone marrow may be observed in many reactive or clonal disorders, the result of which ...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1997
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199705000-00096