Immunodeficiency Flashcards

(61 cards)

1
Q

what is a immunodeficiency

A

absence or failure of normal function of immune syst

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2
Q

what does immunodeficiency do in terms of infection

A

increases susceptibility

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3
Q

what is specific and non specific immundef

A

sp - inv abnormalities of B/T cells

non sp - inv abnormalities in complement or phagocytes

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4
Q

what is primary and secondary immunodef

A

primary - intrinsic defect, genetic, age

secondary - extrinsic factors

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5
Q

what are some extrinsic factors that cause secondary immunodef

A
drug therapies for cancer or autoimmune 
irradiation
organ/bone marrow
malnutrition
infections
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6
Q

what happens in b cell immunodef

A

low serum Ig

recurrent pyogenic infections

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7
Q

what do most b immunodefs occur as

A

primary and rare

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8
Q

how do u treat b cell immunodef

A

IV replacement therapy with Ig

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9
Q

what are some pyogenic infections

A

pneumonia
sinusitis
otis media

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10
Q

what is x linked agamma-globulinaemia

A

No Ig prod
resp infections
sepsis from abscessed teeth

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11
Q

what is common variable immunodeficiency

A

severely decreased or no IgG prod
resp infections
sepsis rm abscessed teeth
asymptomatic

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12
Q

what is selective IgA def

A
sev dec or no IgA prod 
resp and GI infections 
candidiasis 
oral ulcerations
asymptotic
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13
Q

what are t cell immunodef

A

pt’s with no T cell or poor T cell function are susceptible to opportunistic infections - candida or herpes

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14
Q

what are some causes of t cel def

A

MHC def
CD40 ligand def
CD3 mutations
dec T cell num

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15
Q

what is CD40 ligand

A

mol on activated T cells necessary fro APC-T cell interactions

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16
Q

what are T cell immunodef result of

A

humoral def

b cell function dept on T cell function

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17
Q

what are primary t cell immunodef’s

A

rare nd often fatal

severe combined immunodef

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18
Q

what happens to pt with SCID

A

fail to thrive
repeated infections
first 6-9 moth of acquired Ab have some protection thereafter bacterial infections much more common

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19
Q

what are some repeated infections that happen to pt with SCID

A

candida
varicella
herpes simplex
cytomegalovirus

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20
Q

what are some oral manifestations of SCID

A

candidiasis
herpes infections
recurrent ulcerations of tongue and buccal mucosa

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21
Q

what is AIDS

A

acquired immunodef syndrome

acquired immune 
def syndrome
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22
Q

what kind od cell is immunodef in AIDS

A

t cell immunodef

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23
Q

what causes AIDS

A

infection with HIV (human immunodef Virus)

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24
Q

what is aids transmitted by

A
sexual contact 
transfer via placenta or milk mother to infant 
blood transfusion 
needle sharing 
needlestick injury
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25
what are the symptoms with infection of HIV
``` many individuals asymptomatic develop transient fever stolen lymph nodes sore throat rash ```
26
how long does body take to have response against HIV
Abs ag HIV proteins take 2-6 weeks develops
27
what happens after infection of HIV
period of latency up to 2-15 years
28
when s infection with HIV alssfiied as aids
CD4 + T cells <200 cell/uL
29
what are some consequences of aids
major opportunistic infections malignancy thrombocytopenia nervous system diseases
30
what are some major opportunistic infections assc with aids
pneumocytosis mycobacterium cytomegalovirus
31
what malignancy assc with aids
kaposi's sarcoma - tumour of endothelial cells
32
what is thrombocytopenia
low platelet count
33
what re some nervous system diseases assc with aids
dementia paralysis end stage of aids
34
what can control HIV
highly active antiretroviral therapy (HAART)
35
what is HAART composed of
nucleoside analog reverse transcription inhibitors and non- nucleoside RT inhibitor or protease inhibitor
36
when is HAART recommended
when CD4 + T cell count is <350 cell/uL
37
what does HIV/AIDS do to oral health
``` herpes hairy leukoplakia oral candidiasis pathos ulcers oral warts dry mouth ```
38
when does defects in complement proteins occur
rare often inherited
39
what is complement imp for
fighting infection and dissolving immune complexes
40
what do complement def present as
recurrent infections | immune complex disease
41
what is AR
autosomal recessive
42
what is AD
autosomal dominant
43
what causes hereditary angioedema (HAE)
def in main inhibit of classical path , C1 inhib
44
if HAE what else can come later in life
autoantibody | B cell leukemias
45
what are the symptoms of HAE
recurrent episode of angioedema
46
where ca HAE occur
intestine periphery upper airways
47
what happens with intestine HAE
pain cramps vomit
48
what happens in periphery HAE
unsightly swelling
49
what happens in upper airway HAE
resp obstruction which can be fatal
50
what are long term treatments fro HAE
danazol antifibrinolytic agents C1 inhibitor
51
when can oedema occur
spontaneously or after very slightly trauma e.g. minor dental inevrentiosn
52
what can be done in rapid action to maintain an open airway
C1 inhib conc resolve oedema in 30 mins- 2 hours | daily danazol
53
wha are 3 imp genetic defects in phagocyte
chronic granulations disease leucocyte adhesion def chedik-higashi syn
54
how many people does chronic granulomatous disease effect
1-4 ppl in million defective gene X chromo most boys
55
how is granuloma formed mouth
defective NADPH oxidase = anions usually help phagocyte cause cell mandated res = granuloma
56
how to treat chronic granulomatous disease
prophylactic daily antibiotics and IFNy
57
what causes leukocyte adhesion def
lack integrin subunit, imp in adhesion and homing
58
what does leukocyte adhesion deficiency result in
repeated pyogenic infections
59
what are the oral manifestations of leukocyte adhesion def
early childhood preidontitis oral ulcerations delayed wound healing
60
when does chediak-higashi syndrome occur
thro rare autosomal recessive disorder due to mutation in gene that regulates lysosome traffic
61
what is a result of chediak-higashi syndrome
result in giant lysosomal granules in neutrophils, monocytes and lymphocytes infections granuloma form