Immunodeficiency Flashcards

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
Q

what are the symptoms with infection of HIV

A
many individuals asymptomatic 
develop transient fever
stolen lymph nodes
sore throat 
rash
26
Q

how long does body take to have response against HIV

A

Abs ag HIV proteins take 2-6 weeks develops

27
Q

what happens after infection of HIV

A

period of latency up to 2-15 years

28
Q

when s infection with HIV alssfiied as aids

A

CD4 + T cells <200 cell/uL

29
Q

what are some consequences of aids

A

major opportunistic infections
malignancy
thrombocytopenia
nervous system diseases

30
Q

what are some major opportunistic infections assc with aids

A

pneumocytosis
mycobacterium
cytomegalovirus

31
Q

what malignancy assc with aids

A

kaposi’s sarcoma - tumour of endothelial cells

32
Q

what is thrombocytopenia

A

low platelet count

33
Q

what re some nervous system diseases assc with aids

A

dementia
paralysis
end stage of aids

34
Q

what can control HIV

A

highly active antiretroviral therapy (HAART)

35
Q

what is HAART composed of

A

nucleoside analog reverse transcription inhibitors and non- nucleoside RT inhibitor or protease inhibitor

36
Q

when is HAART recommended

A

when CD4 + T cell count is <350 cell/uL

37
Q

what does HIV/AIDS do to oral health

A
herpes 
hairy leukoplakia 
oral candidiasis 
pathos ulcers 
oral warts 
dry mouth
38
Q

when does defects in complement proteins occur

A

rare often inherited

39
Q

what is complement imp for

A

fighting infection and dissolving immune complexes

40
Q

what do complement def present as

A

recurrent infections

immune complex disease

41
Q

what is AR

A

autosomal recessive

42
Q

what is AD

A

autosomal dominant

43
Q

what causes hereditary angioedema (HAE)

A

def in main inhibit of classical path , C1 inhib

44
Q

if HAE what else can come later in life

A

autoantibody

B cell leukemias

45
Q

what are the symptoms of HAE

A

recurrent episode of angioedema

46
Q

where ca HAE occur

A

intestine
periphery
upper airways

47
Q

what happens with intestine HAE

A

pain
cramps
vomit

48
Q

what happens in periphery HAE

A

unsightly swelling

49
Q

what happens in upper airway HAE

A

resp obstruction which can be fatal

50
Q

what are long term treatments fro HAE

A

danazol
antifibrinolytic agents
C1 inhibitor

51
Q

when can oedema occur

A

spontaneously or after very slightly trauma e.g. minor dental inevrentiosn

52
Q

what can be done in rapid action to maintain an open airway

A

C1 inhib conc resolve oedema in 30 mins- 2 hours

daily danazol

53
Q

wha are 3 imp genetic defects in phagocyte

A

chronic granulations disease
leucocyte adhesion def
chedik-higashi syn

54
Q

how many people does chronic granulomatous disease effect

A

1-4 ppl in million
defective gene X chromo
most boys

55
Q

how is granuloma formed mouth

A

defective NADPH oxidase = anions usually help phagocyte cause cell mandated res = granuloma

56
Q

how to treat chronic granulomatous disease

A

prophylactic daily antibiotics and IFNy

57
Q

what causes leukocyte adhesion def

A

lack integrin subunit, imp in adhesion and homing

58
Q

what does leukocyte adhesion deficiency result in

A

repeated pyogenic infections

59
Q

what are the oral manifestations of leukocyte adhesion def

A

early childhood preidontitis
oral ulcerations
delayed wound healing

60
Q

when does chediak-higashi syndrome occur

A

thro rare autosomal recessive disorder due to mutation in gene that regulates lysosome traffic

61
Q

what is a result of chediak-higashi syndrome

A

result in giant lysosomal granules in neutrophils, monocytes and lymphocytes
infections granuloma form