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

Immunodeficiency syndromes (complete) Flashcards

1
Q

What are immunodeficiency diseases

A

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

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2
Q

what are the two categories of immunodeficiency

A

primary and acquired

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3
Q

what is primary immunodeficiency

A

defects to immune components that are genetic

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4
Q

what is acquired immunodeficiency

A

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

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5
Q

what are the three types of immunodeficiency

A

humoral
cellular
combined

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6
Q

what is the main problem with immunodeficiency

A

increased susceptibility to infection

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7
Q

what is usually defective in primary immunodeficiency

A

missing enzyme
missing cell type
non-functioning component

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8
Q

can primary immunodeficiency be aquired

A

yes

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9
Q

can secondary (acquired) immunodeficiency be congenital

A

no

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10
Q

what are the four types of humoral immunodeficiencies

A

Hypogammaglobulinemia
Agammaglobulinemia
Hyper-IgM syndrome
selective IgA deficiency

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11
Q

what is hypogammaglubulinemia

A

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

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12
Q

what is transient hypogammaglobulinemia of infancy

A

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

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13
Q

how is transient hypogammalobulinemia of infancy combatted

A

the maternal IgG confers protection to the infant

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14
Q

at what point does transient hypogammaglobuilinemia of infancy resolve itself

A

at around 9 months, the infants develops its own Ab

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15
Q

What is agammaglobulinemia

A

no antibodies in the blood

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16
Q

what is bruton’s agammaglobulinemia

A

a deficiency in circulating mature B-cells

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17
Q

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

A

mutation/deletion of Bcell tyrosine kinase

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18
Q

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

A

normal

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19
Q

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

A

when the mothers IgG declines

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20
Q

is bruton’s agammaglobulinemia primary or secondary

A

primary (x-linked)

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21
Q

is transient hypogammaglobuilinemia of infancy primary or secondary

A

primary

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22
Q

how is bruton’s agammaglobulinemia treated

A
  1. prevention of infection with vaccination (dead vaccines only)
  2. agressive antibiotic treatment of infections
  3. monthly injections of intravenous immunoglobin
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23
Q

What is hyper-IgM syndrome

A

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

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24
Q

is hyper-IgM syndrome primary or secondary

A

primary (x-linked)

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25
Q

what are the defects that lead to hyper-IgM syndrome

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

2. defects in AID

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26
Q

what are they symptoms of hyper-IgM syndrome

A

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

27
Q

how is hyper-IgM syndrome treated

A

monthly Intravenous immunoglobin (IVIg) injections

28
Q

What is the most common immunodeficiency

A

IgA deficiency

29
Q

what is IgA deficiency

A

low IgA levels

30
Q

what are the symptoms of someone with IgA deficiency

A

normal, respiratory infections, diarrhea

31
Q

what does IgA deficiency predispose someone to

A
  1. celiac disease
  2. oral mucosal infections

it doesn’t affect periodontal or dental health

32
Q

is selective immunoglobin isotope deficiency primary or secondary

A

primary

33
Q

what is CVID

A

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

34
Q

which cell type is absent in CVID

A

plasma cells

35
Q

how do you diagnose CVID

A

rule out all other types of primary immunodeficiency

36
Q

is CVID primary or secondary immunodeficiency

A

primary

37
Q

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

A

low IgG, and IgA

normal/reduced IgM and B-cells

38
Q

are malignant tumors common in patients with CVID

A

yes

39
Q

What are the different types of cellular immunodeficiencies

A

DiGeorge syndrome

40
Q

what is DiGeorge syndrome

A

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

41
Q

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

A

normal B-cell levels

low or absent T-cells

42
Q

what is the thymus like inDiGeorge syndrome

A

small

43
Q

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

A

viral infections.

44
Q

how do you treat someone with DiGeorge syndrome

A

transplantation with a fetal thymus

45
Q

What is SCID

A

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

46
Q

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

A

both are very low

47
Q

what is the boy in the disorder that is associated with the “boy in the plastic bubble”

A

SCID

48
Q

how does a patient get SCID

A

x-linked inherited

49
Q

what is the treatment for someone with SCID

A

bone marrow transplant

50
Q

what is CGD

A

chronic granulomatus disorder, a defect in phagocytes

51
Q

what is the defect in the phagocytes that causes CGD

A

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

52
Q

what are the common infections that accompany CGD

A

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

53
Q

how is CGD treated

A

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

54
Q

What can cause immunodeficiency

A

malnutrition, cancer, or infection (HIV)

55
Q

what is acquired immunodeficiency syndrome

A

AIDS

56
Q

what causes AIDS

A

HIV

57
Q

how does HIV lead to immunodeficiency

A

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
Q

how do you detect HIV

A

anti-HIV Ab detection

59
Q

how is HIV treated

A

triple drug therapy (HAART)

highly active anti-ertroviral therapy

60
Q

what does HIV ultimately lead to

A

depletion of CD4+ cells = AIDS

CD4 cells are 1/10 the normal amount

61
Q

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

A

gp120

62
Q

can HIV lead to periodontal disease

A

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

63
Q

what are the types of oral manifestations of HIV

A 
aphthous ulceration
oral hairy leukoplakia
oral candidiasis
necrotizing ulcerative periodontitis
necrotizing ulcerative stomatitis
(risk of PDL attachment loss and gingival recession)
64
Q

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

A

oral candidiasis (white lesions on the palate)

DMD 5244 (Immunity) (16 decks)

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