🖋️ Notes: Y1 i&d - immuno deficiency

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Y1, I&D, IND, infection and defence, I+D, I and D, y1Tcells, Y1Bcells, Benny Chain I&D:

X-linked agammaglobulinaemia (Bruton’s Disease)- no B cells produced
o Di George Syndrome- no T cells produced from thymus
o Wiskott-Aldrich Syndrome- defect in cytoskeleton in lymphocytes and
NK cells
o X-linked hyper IgM syndrome- no B cells class switching of IgM (no
secondary immune response)
o X-linked lymphoproliferative disease- stops apoptosis of cells
(susceptible to Epstein-Barr virus)
o Severe Combined Immunodeficiency (SCID)- cellular and humoral
immunity is affected
Secondary Immunodeficiencies:

o due to external agents affecting the immune system
o more common than primary immunodeficiencies
o Examples:
▪ Malnutrition- lack dietary elements
▪ Loss of cellular/ humoral components- lose antibodies in urine
(nephrotic syndrome), lymphocytes lost in intestine (intestinal
lymphangiectasis)
▪ Tumours- bad for cells and immune system
▪ Cytotoxic drugs/ irradiation- damage immune cells
▪ Diabetes-associated with infection
▪ Infections- inhibits immune response (HIV, malaria, leukaemia)

Treatment:

o antibiotic therapy- fight pathogen
o Passive gammaglobulin therapy- used when antibody deficiencies
o Bone marrow and haematopoietic stem cell transplantation- new source
of immune cells
o gene therapy- replace faulty genes with normal genes (used in SCID)