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Hypogammaglobulinemia is an immune system disorder in which not enough gamma globulins are produced in the blood (thus hypo-+ gamma + globulin + -emia). This results in a lower antibody count, which impairs the immune system , increasing risk of infection.
IgG deficiency is often found in children as transient hypogammaglobulinemia of infancy, which may occur with or without additional decreases in IgA or IgM. IgG subclass deficiencies are also an integral component of other well-known primary immunodeficiency diseases, such as Wiskott–Aldrich syndrome and ataxia–telangiectasia.
It is unknown what specifically causes infantile transient hypogammaglobulinemia. The following are some of the hypothesized mechanisms: 1) defective T cells that prevent B cells from stimulating the proper synthesis of antibodies; 2) maternal IgG suppresses the production of IgG; 3) low levels of vital cytokines; and 4) genetic variations in families predisposed to immunodeficiency.
Infusions of immune globulin can reduce the frequency of bacterial infections, and G-CSF or GM-CSF therapy improves blood neutrophil counts. [5]As WHIM syndrome is a molecular disease arising from gain-of-function mutations in CXCR4, preclinical studies identified plerixafor, a specific CXCR4 antagonist, as a potential mechanism-based therapeutic for the disease. [6]
Patent term extensions were offered to manufacturers that conducted trials of drugs that would be used in children. The Pediatric Research Equity Act of 2003, Congress codified the FDA's authority to mandate manufacturer-sponsored pediatric drug trials for certain drugs as a "last resort" if incentives and publicly funded mechanisms proved ...
Common variable immunodeficiency; Specialty: Immunology : Symptoms: Hypogammaglobulinemia, recurrent opportunistic infections, fatigue: Complications: Autoimmune manifestations; increased risk of malignancies such as gastric carcinomas, non-Hodgkin lymphoma; lymphocytic infiltration of tissues; nodular regenerative hyperplasia; enteropathy
Expecting that individuals with such a history of repeated infections would have high levels of infection-fighting antibodies in their serum, Dr. Bruton was surprised to find that the boy had hypogammaglobulinemia, i.e., his serum lacked detectible levels of circulating antibodies which attack infection-causing microorganisms and virus.
Selective immunoglobulin A (IgA) deficiency (SIgAD [1]) is a kind of immunodeficiency, a type of hypogammaglobulinemia.People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract.