EX2 Immunodeficiency Diseases - Powell Flashcards Preview

SP14 Microbiology > EX2 Immunodeficiency Diseases - Powell > Flashcards

Flashcards in EX2 Immunodeficiency Diseases - Powell Deck (30):
1

This type of immunodeficiency is caused by genetic defects that result in an increased susceptibility to infection

primary or congenital immunodeficiency

2

What are the four main factors that can lead to secondary or acquired immunodeficiency

malnutrition
disseminated cancer
immunosuppressive drugs
infection of immune system cells

3

Which type of immunodeficiency are frequently manifested in children and affect about 1/500 people in the USA

primary or congenital immunodeficiency

4

This is vital for the defense against infectious organisms and their toxic products

the integrity of the immune system

5

These are conserved across widely diverse species and any loss-of-function mutation has negative consequences for survival

Toll-like receptors

6

True or False
primary immunodeficiencies may affect one or more components of the immune system (T-cells, B-cells, NKs, etc.)

True

7

With primary immunodeficiency, it may result from what defects

defects in leukocyte maturation or activation or from defects in effector mechanisms of innate and adaptive immunity

8

What is the principal consequence of an immunodeficiency disease

increased susceptibility to infection

9

The nature of the infection in a particular patients depends largely on what

the component of the immune system that is defective

10

A deficient humoral immunity will result in what

increased susceptibility to infection by pyogenic bacteria

11

With X-linked Agammaglobulinemia, this is very low, these are usually absent, and these are present but in reduced numbers

all antibody isotopes are very low
circulating B cells are usually absent
pro-B cells are present but reduced numbers in bone marrow

12

What physical attribution are associated with X-linked agammaglobulinemia

tonsils are very small
lymph nodes are barely palpable
thymus is normal (as are other T cell centers)

13

X-linked agammaglobulinemia's defect is associated with

loss of function of the Bruton Tyrosine Kinase that is important for pre-B cell expansion and maturation into Ig-expresion B cells

14

Deficient cell-mediated immunity will result in what

increased susceptibility to viruses and other intracellular pathogens

15

True or False
There are many different treatments for defects associated with deficient T cell responses

False; there are few, if any treatments

16

True or False
It is rare that patients with absolute defects in T-cell function survive beyond infancy or childhood

True

17

DiGeorge's syndrome is a developmentally-related disease where what fails to develop

the thymus

18

What is increased in DiGeorge's syndrome, and also impaired

the percentage of B cells increases, but the function is impaired only to the extent of needing helper T cells

19

What is the outcome of patients with DiGeorge's syndrome

most infants die from infections, and those who make it past infancy usually develop some kind of mental retardation

20

True or False
Severe combined immunodeficiency (T cell and B cell deficiencies) are rare and fatal

True

21

True or False
X-linked SCID (XSCID) is the most common x-linked recessive severe combined immunodeficiency disease, accounting for approximately 42% of cases

True

22

X-linked SCID patients develop what and lack this

develop persistent infection with opportunistic organisms
lack the ability to reject foreign tissues; at risk for GVHD

23

X-linked SCID patients have few or no what (2), but an elevated percentage of this

T cells and NK cells
B cells (but they do not produce Ig)

24

What are the aims of immunodeficiency treatments

to minimize and control infections
to replace the defective or absent components of the immune system by adoptive transfer/transplantation

25

This is a valuable treatment for agammaglobulinemic patients, and has been life saving

passing immunization with pooled gamma globulin

26

This is the current treatment of choice for various immunodeficiency diseases; and has been successful for SCID and similar diseases

bone-marrow transplantation

27

The course of HIV after the primary infection and immune response is what

clinical latency (establishment of chronic infection, low-level viral production)

28

After HIV is deem clinically latent, what then happens after other microbial infections

there is increased viral replication

29

After HIV undergoes rapid replication, what occurs

AIDS; destruction of lymphoid tissues, depletion of CD4 T cells, and death

30

True or False
HIV doesn't cause immunodeficiency, it causes AIDS which does

True