Immunodeficiency Flashcards
what is a immunodeficiency
absence or failure of normal function of immune syst
what does immunodeficiency do in terms of infection
increases susceptibility
what is specific and non specific immundef
sp - inv abnormalities of B/T cells
non sp - inv abnormalities in complement or phagocytes
what is primary and secondary immunodef
primary - intrinsic defect, genetic, age
secondary - extrinsic factors
what are some extrinsic factors that cause secondary immunodef
drug therapies for cancer or autoimmune irradiation organ/bone marrow malnutrition infections
what happens in b cell immunodef
low serum Ig
recurrent pyogenic infections
what do most b immunodefs occur as
primary and rare
how do u treat b cell immunodef
IV replacement therapy with Ig
what are some pyogenic infections
pneumonia
sinusitis
otis media
what is x linked agamma-globulinaemia
No Ig prod
resp infections
sepsis from abscessed teeth
what is common variable immunodeficiency
severely decreased or no IgG prod
resp infections
sepsis rm abscessed teeth
asymptomatic
what is selective IgA def
sev dec or no IgA prod resp and GI infections candidiasis oral ulcerations asymptotic
what are t cell immunodef
pt’s with no T cell or poor T cell function are susceptible to opportunistic infections - candida or herpes
what are some causes of t cel def
MHC def
CD40 ligand def
CD3 mutations
dec T cell num
what is CD40 ligand
mol on activated T cells necessary fro APC-T cell interactions
what are T cell immunodef result of
humoral def
b cell function dept on T cell function
what are primary t cell immunodef’s
rare nd often fatal
severe combined immunodef
what happens to pt with SCID
fail to thrive
repeated infections
first 6-9 moth of acquired Ab have some protection thereafter bacterial infections much more common
what are some repeated infections that happen to pt with SCID
candida
varicella
herpes simplex
cytomegalovirus
what are some oral manifestations of SCID
candidiasis
herpes infections
recurrent ulcerations of tongue and buccal mucosa
what is AIDS
acquired immunodef syndrome
acquired immune def syndrome
what kind od cell is immunodef in AIDS
t cell immunodef
what causes AIDS
infection with HIV (human immunodef Virus)
what is aids transmitted by
sexual contact transfer via placenta or milk mother to infant blood transfusion needle sharing needlestick injury
what are the symptoms with infection of HIV
many individuals asymptomatic develop transient fever stolen lymph nodes sore throat rash
how long does body take to have response against HIV
Abs ag HIV proteins take 2-6 weeks develops
what happens after infection of HIV
period of latency up to 2-15 years
when s infection with HIV alssfiied as aids
CD4 + T cells <200 cell/uL
what are some consequences of aids
major opportunistic infections
malignancy
thrombocytopenia
nervous system diseases
what are some major opportunistic infections assc with aids
pneumocytosis
mycobacterium
cytomegalovirus
what malignancy assc with aids
kaposi’s sarcoma - tumour of endothelial cells
what is thrombocytopenia
low platelet count
what re some nervous system diseases assc with aids
dementia
paralysis
end stage of aids
what can control HIV
highly active antiretroviral therapy (HAART)
what is HAART composed of
nucleoside analog reverse transcription inhibitors and non- nucleoside RT inhibitor or protease inhibitor
when is HAART recommended
when CD4 + T cell count is <350 cell/uL
what does HIV/AIDS do to oral health
herpes hairy leukoplakia oral candidiasis pathos ulcers oral warts dry mouth
when does defects in complement proteins occur
rare often inherited
what is complement imp for
fighting infection and dissolving immune complexes
what do complement def present as
recurrent infections
immune complex disease
what is AR
autosomal recessive
what is AD
autosomal dominant
what causes hereditary angioedema (HAE)
def in main inhibit of classical path , C1 inhib
if HAE what else can come later in life
autoantibody
B cell leukemias
what are the symptoms of HAE
recurrent episode of angioedema
where ca HAE occur
intestine
periphery
upper airways
what happens with intestine HAE
pain
cramps
vomit
what happens in periphery HAE
unsightly swelling
what happens in upper airway HAE
resp obstruction which can be fatal
what are long term treatments fro HAE
danazol
antifibrinolytic agents
C1 inhibitor
when can oedema occur
spontaneously or after very slightly trauma e.g. minor dental inevrentiosn
what can be done in rapid action to maintain an open airway
C1 inhib conc resolve oedema in 30 mins- 2 hours
daily danazol
wha are 3 imp genetic defects in phagocyte
chronic granulations disease
leucocyte adhesion def
chedik-higashi syn
how many people does chronic granulomatous disease effect
1-4 ppl in million
defective gene X chromo
most boys
how is granuloma formed mouth
defective NADPH oxidase = anions usually help phagocyte cause cell mandated res = granuloma
how to treat chronic granulomatous disease
prophylactic daily antibiotics and IFNy
what causes leukocyte adhesion def
lack integrin subunit, imp in adhesion and homing
what does leukocyte adhesion deficiency result in
repeated pyogenic infections
what are the oral manifestations of leukocyte adhesion def
early childhood preidontitis
oral ulcerations
delayed wound healing
when does chediak-higashi syndrome occur
thro rare autosomal recessive disorder due to mutation in gene that regulates lysosome traffic
what is a result of chediak-higashi syndrome
result in giant lysosomal granules in neutrophils, monocytes and lymphocytes
infections granuloma form
