A 5-year-old child is diagnosed with Bruton's discuso (X-linked agammaglobulinemia) that manifests itself in severe clinical course of bacterial infections and absence of B lymphocytes and plasma cells. What changes of immunoglobulin content can be observed in blood serum of the child with immunodeficiency?
Answer options
- No changes
- Increased IgA. IgM
- Decreased IgA,IgE
- Increased IgD, IgE
- Decreased IgA,IgM
Decreased IgA,IgM
Explanation
Primary humoral immunodeficiency characterized by decreased immunoglobulins Genetics X-linked recessiveseen in male children Pathogenesis Defect in Bruton tyrosine kinase (BTK) defective maturation of B- cells impaired signaling from pre-B-cell receptor ↓ B-cells ↓ production of all classes of Ig impaired antibody immune response Perform initial studies measuring quantitative IgG, IgM, immunoglobulin E (IgE), and immunoglobulin A (IgA). IgG levels below 100 mg/dL are usually indicative of X-linked agammaglobulinemia (XLA). The detection of IgG, IgA, IgM, and IgE levels is related to age. Typically, IgM and IgA are undetectable. All levels are reduced in males with XLA. Age-specific reference range values are available to compare with the patient's level.X-linked agammaglobulinemia (XLA)?X-linked agammaglobulinemia (a-gam-uh-glob-u-lih-NEE-me-uh) — or XLA — is an inherited (genetic) immune system disorder that reduces the ability to fight infections. People with XLA may experience infections of the inner ear, sinuses, respiratory tract, bloodstream, and internal organs.XLA affects males exclusively, although females can be genetic carriers of the condition. Diagnosis of XLA often occurs in infancy or early childhood once it's clear that a child has experienced repeated infections. Sometimes, the diagnosis doesn't occur until adulthood.
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