Immunodeficiency syndromes (complete) Flashcards Preview

DMD 5244 (Immunity) > Immunodeficiency syndromes (complete) > Flashcards

Flashcards in Immunodeficiency syndromes (complete) Deck (64):
1

What are immunodeficiency diseases

diseases caused by defects to one or more of the components of the immune system

2

what are the two categories of immunodeficiency

primary and acquired

3

what is primary immunodeficiency

defects to immune components that are genetic

4

what is acquired immunodeficiency

defects to immune components that are due to infection, immunosupressive drugs or malnutrition

5

what are the three types of immunodeficiency

humoral
cellular
combined

6

what is the main problem with immunodeficiency

increased susceptibility to infection

7

what is usually defective in primary immunodeficiency

missing enzyme
missing cell type
non-functioning component

8

can primary immunodeficiency be aquired

yes

9

can secondary (acquired) immunodeficiency be congenital

no

10

what are the four types of humoral immunodeficiencies

Hypogammaglobulinemia
Agammaglobulinemia
Hyper-IgM syndrome
selective IgA deficiency

11

what is hypogammaglubulinemia

when you don't have enough of a certain Immunoglobin/antibody

12

what is transient hypogammaglobulinemia of infancy

a normal phenomenon, in which an infant doesn't have enough antibody/Ig. this is caused by a delay in B-cell development

13

how is transient hypogammalobulinemia of infancy combatted

the maternal IgG confers protection to the infant

14

at what point does transient hypogammaglobuilinemia of infancy resolve itself

at around 9 months, the infants develops its own Ab

15

What is agammaglobulinemia

no antibodies in the blood

16

what is bruton's agammaglobulinemia

a deficiency in circulating mature B-cells

17

what causes bruton's agammaglobulinemia's shortage or mature B-cells

mutation/deletion of Bcell tyrosine kinase

18

what are the T-cells like in bruton's agammaglobulinemia

normal

19

when do you start to see infectious symptoms in bruton's agammaglobulinemia

when the mothers IgG declines

20

is bruton's agammaglobulinemia primary or secondary

primary (x-linked)

21

is transient hypogammaglobuilinemia of infancy primary or secondary

primary

22

how is bruton's agammaglobulinemia treated

1. prevention of infection with vaccination (dead vaccines only)
2. agressive antibiotic treatment of infections
3. monthly injections of intravenous immunoglobin

23

What is hyper-IgM syndrome

when B-cells can't do class switching, so you only have IgM

24

is hyper-IgM syndrome primary or secondary

primary (x-linked)

25

what are the defects that lead to hyper-IgM syndrome

1. defects in CD40 on T-cell or CD40L on B-cell
2. defects in AID

26

what are they symptoms of hyper-IgM syndrome

predisposed to bacterial infections, fungal infection, pneumocytis jiroveci, and other opportunistic infections

27

how is hyper-IgM syndrome treated

monthly Intravenous immunoglobin (IVIg) injections

28

What is the most common immunodeficiency

IgA deficiency

29

what is IgA deficiency

low IgA levels

30

what are the symptoms of someone with IgA deficiency

normal, respiratory infections, diarrhea

31

what does IgA deficiency predispose someone to

1. celiac disease
2. oral mucosal infections

it doesn't affect periodontal or dental health

32

is selective immunoglobin isotope deficiency primary or secondary

primary

33

what is CVID

common variable immunodeficiency, a group of disorders that lead to low Ab levels

34

which cell type is absent in CVID

plasma cells

35

how do you diagnose CVID

rule out all other types of primary immunodeficiency

36

is CVID primary or secondary immunodeficiency

primary

37

what are the levels of IgG, IgA, IgM, and b-cells in patients with CVID

low IgG, and IgA
normal/reduced IgM and B-cells

38

are malignant tumors common in patients with CVID

yes

39

What are the different types of cellular immunodeficiencies

DiGeorge syndrome

40

what is DiGeorge syndrome

a primary immunodeficiency where there is a defect in t-cell maturation

41

what are the B and T-cell levels in DiGeorge syndrome

normal B-cell levels
low or absent T-cells

42

what is the thymus like inDiGeorge syndrome

small

43

what type of infection is most common to those with DiGeorge syndrome

viral infections.

44

how do you treat someone with DiGeorge syndrome

transplantation with a fetal thymus

45

What is SCID

severe, combined immunodeficiency, a primary immunodeficiency in which both B and T-cells are very low

46

what are the levels of B and T-cells like in SCID

both are very low

47

what is the boy in the disorder that is associated with the "boy in the plastic bubble"

SCID

48

how does a patient get SCID

x-linked inherited

49

what is the treatment for someone with SCID

bone marrow transplant

50

what is CGD

chronic granulomatus disorder, a defect in phagocytes

51

what is the defect in the phagocytes that causes CGD

they can't produce H2O2 or O2 due to a genetic defect in NADPH oxidase

52

what are the common infections that accompany CGD

pneumonia, lymph node infection, lung infection, abscesses on skin or liver

53

how is CGD treated

with long term antibiotics and IFN-y injections to activate macrophages

54

What can cause immunodeficiency

malnutrition, cancer, or infection (HIV)

55

what is acquired immunodeficiency syndrome

AIDS

56

what causes AIDS

HIV

57

how does HIV lead to immunodeficiency

it binds to CD4 on T-cells, and replicates within them. then it is processed and presented to cytotoxic T-cells, and the Cytotoxic T-cells kill off the CD4 T-cells

58

how do you detect HIV

anti-HIV Ab detection

59

how is HIV treated

triple drug therapy (HAART)
highly active anti-ertroviral therapy

60

what does HIV ultimately lead to

depletion of CD4+ cells = AIDS
CD4 cells are 1/10 the normal amount

61

what part of HIV binds to the CD4 of T-cells

gp120

62

can HIV lead to periodontal disease

yes (especially those with a high viral load and low CD4 T-cell counts)

63

what are the types of oral manifestations of HIV

aphthous ulceration
oral hairy leukoplakia
oral candidiasis
necrotizing ulcerative periodontitis
necrotizing ulcerative stomatitis
(risk of PDL attachment loss and gingival recession)

64

what is a marker of mild immunodeficiency in the mouth, and is often the first manifestation of an HIV infected patient

oral candidiasis (white lesions on the palate)