An Adult Diagnosed as Hyper-IgM Immunodeficiency Syndrome.
نویسندگان
چکیده
منابع مشابه
Hyper IgM Syndrome with low IgM and thrombocytosis: an unusual case of immunodeficiency.
We report a 5 years old male child with low serum IgG, IgA and IgM levels, who presented with recurrent perianal and oral ulcers, intermittent fever, and protracted diarrhea. Despite the lack of typical respiratory symptoms, low serum IgM level and persistent thrombocytosis, an X-linked hyper-IgM syndrome (X-HIGM) was considered. Laboratory investigations revealed a diagnosis of hyper-IgM syndr...
متن کاملHyper-IgM Immunodeficiency with Enamel Defects: a Case Report
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...
متن کاملHyper IgM syndrome presenting as chronic suppurative lung disease
The Hyper-immunoglobulin M syndromes (HIGM) are a heterogeneous group of genetic disorders resulting in defects of immunoglobulin class switch recombination. Affected patients show humoral immunodeficiency and high susceptibility to opportunistic infections. Elevated serum IgM levels are the hallmark of the disease, even though in few rare cases they may be in the normal range. Hyper IgM is ass...
متن کاملHyper-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....
متن کاملCD154 and type-1 cytokine response: from hyper IgM syndrome to human immunodeficiency virus infection.
CD40 and CD154 (CD40 ligand) are surface molecules that are central to the cross-talk between T cells and antigen-presenting cells. This article reviews the relevance of CD40-CD154 interaction for regulation of interleukin-12/interferon-gamma production in response to Toxoplasma gondii as an example of an intracellular pathogen. The manner in which defects in CD154 signaling contribute to immun...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Internal Medicine
سال: 1995
ISSN: 0918-2918,1349-7235
DOI: 10.2169/internalmedicine.34.640