Please enable JavaScript.

Coggle requires JavaScript to display documents.

IMMUNODEFICIENCY DISORDER -disorder of immune sys tthat result in…

- - - - B Cells 50%
        
        B cell disorders
        
        b. Selective IgA Deficiency
        
        Most common of immunodeficiency disorders
        
        B cells do not differentiate to IgA secreting plasma cells
        
        2 factor which is genetic defect but not known the effect will occut yet second is clinical features which can Increased incidence of recurrent sino-pulmonary infection.
        
        c. Immunoglobulin Deficiency with increased IgM
        
        Disorder with an increase level of serum IgM but decrease in IgG, IgA and IgE
        
        Appears to be X linked
        
        2 factor which is genetic defect will Defect CD40L and clinical features which is Recurrent and severe bacterial infections
        
        B cells number may be decrease or maybe normal but their function is impaired
        
        Individuals with antibody defects will present clinically with recurrent bacterial infections
        
        have involve one or more antibody isotypes
        
        a. X-linked Agammaglobulinemia (XLA)
        
        An immunodeficiency disorder linked to the X chromosome
        
        male (XY) who inherit the defective gene (X chromosome)
        
        will present with this disorder
        
        have 2 factor which is Genetic defect which is Result in a single gene that code thyrosine kinase and second is Clinical features which is mother antibodies that across the placenta have been degraded
      - Combined B & T cell 15-20%
      - Phagocytes 20%
      - Complement 2-4%
      - T Cell 10%
        
        T cell disorder
        
        ] Combined T cell and B cell disorders
        
        defects in the bone marrow progenitor cells
        
        give arise to T cell and B cells population.
        
        have 2 type disorder
        
        a. Severe combined immunodeficiency disorders (SCID)Link Title
        
        1 more item...
        
        defect in bone marrow progenitor cells
        
        Defect in signaling molecules or enzymes
        
        genetic defect have About 50% of identified cases of SCID are due to defect in enzyme adenosine deaminase (ADA)
        
        Both male and female can be affected
        
        have one syndrome called DiGeorge’s syndrome
        
        a. DiGeorge’s syndrome
        
        referred as conginetal thymic hypoplasia
        
        Result from defective embryogenesis leading to a reduce and defective (or absent) thymus
        
        have 2 factor , first genetic defect which is Associated with defects on chromosome 22.
        
        second is clinical features
        
        present with increase susceptibility to viral, fungal and intracellular bacterial infections
        
        have physical malformations arising from developmental defects