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

Immunodeficiency syndromes (complete) Flashcards

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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
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)

DMD 5244 (Immunity) (16 decks)

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