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Flashcards in Immunodeficiency Diseases - Powell Deck (21)
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1

What is a Immunodeficiency?

Basically when, part of your immune system is missing or dysfunctional.

2

What are the 2 main types of immunodeficiencies?

Primary or congenital

Secondary or acquired immunodeficiencies

3

What is Primary immunodeficiency?

Are genetic defects that result in an increased susceptibility to infection.
Is frequently manifested infancy and childhood
*affects about 1 in 500 people

4

What is Secondary Immunodeficiency?

Develop as a consequence of:
-Malnutrition
- disseminated cancer
- Treatment with immune suppressive therapies
- infection of cells of the immune system.

5

Any loss of function mutation affecting a Toll-like receptor has what kind of effect?

Negative consequences for survival

6

________ disorders may affect one or more components of the immune system, including T & B lymphocytes and Nk cells.

Primary Immunodeficiencies

7

What is the Principal consequence of having an immunodeficiency?

Increased risk of infection

8

The types of _________ can predict the type of immunodeficiency.

type of recurring infection

9

Deficient _________ immunity usually results I'm increased susceptibility to infection by Pyogenic bacteria.

Humoral

10

What are the defining features of XLA (Burton's Agammaglobulinemia)?

- Antibody isotypes are very low - not even IgM or IgD
- Circulating B cells are usually absent
- Pre B cells are reduced in #s in the bone marrow
- Tonsils are small and lymph nodes are barely palpable due to lack of germinal centers.
- Thymus structure is normal
- Most boys affected by XLA remain well during first 6-9 months of life (via maternal transmitted IgG)......Then infections start.

11

_______________ is associated with a loss of function of Bruton Tyrosine Kinase that is important for pre-B cell expansion and maturation into Ig-expressing B cells.

XLA

12

What are the characteristics of X-linked immunodeficiency with Hyper-IgM?

- Very low serum IgG, and IgE
- A elevation concentration of polyconal IgM
* Like XLA, patients may be asymptomatic during first year or two of life.
*In contrast to XLA, patients have Lymphoid hyperplasia.

13

__________ is associated with a loss of function of CD40 ligand that is expressed on helper T cells.

X-linked immunodeficiency with Hyper-IgM

14

What does the loss of CD40 ligand cause?

- Prevents the T cell from stimulating antigen-specify B cells.
- So B cells are not signaled buy T cells to undergo isotope switching and therefore only produce IgM.

15

What is the treatment for Immunodeficiency in Humoral Immunity?

Treatment is routine prophylactic antibiotics and/or gamma-globulin therapy.

16

Deficient ___________ immunity usually results in an increased susceptibility to viruses and other intracellular pathogens.

Cell-mediated immunity

17

What is the treatment for defects associated with Deficient T cell response?

Very few treatments
- rarely survive childhood.

18

What is DiGeorge's Syndrome?

This is a developmentally related disease associated with tissue morphogenesis - The Thymus does not develop!
*The # of T cells is very decreased and infants live months to 2 years.

19

What is X-linked SCID?

It is the most common "X-linked Recessive Severe Combined Immunodeficiency Disease.
*"Bubble Boy" severe combined immunodeficiency, fatal syndrome characterized by profound deficiencies of T & B cell function.

20

What are the 2 goals of treatment of Immunodeficiencies?

1. Minimize and control infections
2. Replace defective part or absent components (passive immunization or bone marrow transplant)

21

What is a prime example of a Secondary or acquired Immunodeficiency?

HIV