Immunodeficiency Flashcards
What is primary immunodeficiency?
Immunodeficiency by genetic defects.
What is secondary (aquired) immunodeficiency
caused by infection, immunosupressant drugs or malnutrition.
What humoral immunodeficiency? Cellular? Combined?Z
H: Anibodies/complement
Cell: T-cells
Combination of both
What is transient hypogammaglobulinemia of infancy?
Infants have initial maternal IgG that protects them. They have a delay in B cell development that decrease Ab levels for first year of life.
What is Brutons Agammaglobulinemia? What is it caused by? What is the treatment?
Circulating mature B cell deficiency caused by an X-linked mutation/deletion of Btk gene. Prevent infection thru vaccination, treat with antibiotics and injections of IVIg.
What is Hyper-IgM syndrome? What is it caused by? Treatment?
B cells cant class switch from IgM caused by an X-linked genetic defect. Treat with Monthly injections of IVIg.
What is Selective Immunoglobulin IgA deficiency? What are some characteristics?
Now serum IgA. Most common Immunodificiency. Celiac disease also common in these patients. Predisposes to oral mucosal infections.
What is Common Variable Immunodificiency (CVID)?
Heterogeneous group of disorders where mature B cells are present, but plasma cells are absent. Have impaired Ab responses to infections or vaccination.
What is DiGeorge Syndrome? Symptoms? Treatment?
Defect in T lymphocyte maturation. Thymus reduced in size with recurrent viral infections. Treatment is transplantation with a fetal thymus.
What is Severe combined immunodificiency (SCID)? how is it inherited? Treatment?
Cell-mediated and Humoral immunity is deficient. X-linked. Treatment with bone marrow transplant.
What is chronic granulomatous disease (CGD)? Patients show a high frequency of what? Treatment?
Failure of phagocyte to elimate pathogens due to NADPH oxidase defects. High frequency of oral complications. Treat with antibiotic and INF-y to activate macrophages.
Acquired Immune Deficiency syndrome (AIDS) is caused by what? What is the mechanism?
HIV infection. HIV binds to CD4 on Th cells. Th cells process and present antigen to CTL, which destroy Th cells leading to low CD4 Th counts.
HIV infected patients are more likely to develop what?
Destructive peridontal infections and other oral manifestations.
Immune suppression has been show to increase what in oral manifestations?
Increase risk of periodontal attachment loss and gingival recession.
