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  4. Selective antibody deficiency with normal immunoglobulins (sadni)

Selective antibody deficiency with normal immunoglobulins (sadni)

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  • Gregoryundefined Offline
    Gregoryundefined Offline
    Gregory
    wrote on last edited by admin
    #1

    Selective antibody deficiency with normal immunoglobulins (Ig) is characterized by deficient specific antibody response to polysaccharide antigens but not to protein antigens, despite normal or near normal serum levels of immunoglobulins, including IgG subclasses.
    (See also Overview of Immunodeficiency Disorders and Approach to the Patient With an Immunodeficiency Disorder).
    Selective antibody deficiency with normal immunoglobulins (SADNI) is a primary immunodeficiency disorder. It is one of the most common immunodeficiencies that manifests with recurrent sinopulmonary infections. Selective antibody deficiencies can occur in other disorders, but SADNI is a primary disorder in which deficient response to polysaccharide antigens is the only abnormality (see table Humoral Immunity Deficiencies). The inheritance and pathophysiology have not been elucidated, but some evidence suggests that the cause may be inherited molecular abnormalities.
    A subset of patients with SADNI initially have an appropriate response to polysaccharide antigen but lose antibody titers within 6 to 8 months (called SADNI memory phenotype).
    Patients have recurrent sinopulmonary infections and sometimes manifestations that suggest atopy (eg, chronic rhinitis, atopic dermatitis, asthma). Severity of the disorder varies.
    Young children may have a form of SADNI that resolves spontaneously over time.

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