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Immunology > Diseases in the Immuncompromised > Flashcards

Diseases in the Immuncompromised Flashcards

1
Q

what is the most common form of SCID

A

X-linked

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2
Q

what defect causes X-linked SCID

A

defect in the gamma chain of the cytokine receptor or Jak3 gene

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3
Q

a lack of which IL prevent T cell development

A

IL-7

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4
Q

which cytokines usually bind the receptor which is defected

A

IL-2 IL-4 IL-7 IL-9 IL-15 IL-21

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5
Q

which activity is affected in X linked SCID

A

cytokine responses

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6
Q

what activities are affected in the various forms of SCID

A

t cell development, lymphocyte survival, vdj rcombination, tcr signalling

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7
Q

what defect causes digeorge syndrome

A

deletion of chromosome 22

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8
Q

what are the symptoms of digeorge syndrome

A

heart defects, cleft palette, autism, other learning difficulties

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9
Q

which actvity is affected in digeorge syndrome

A

thymic development

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10
Q

how does a lack of thymus in Digeorge syndrome cause prblems

A

there are no mature T cells

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11
Q

how do SCID and CID differ

A

in CID there is still some residual function

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12
Q

how is there a decrease in antibodies in Digeorge syndrome

A

a lack of T cells means a lack of b cell activation from t cell dependant antigens

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13
Q

what is the treatment for digeorge syndrome

A

thymic transplant

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14
Q

what type of immunodeficiency is bruntons aggamaglobulineamia

A

humoural immunodeficiency

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15
Q

what process is affected in bruntons aggaaglobulinaemia

A

maturation of b cells

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16
Q

what causes the lack of b cells in bruntons aggamaglobulinaemia

A

lack of Btk in the bone marrow

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17
Q

is bruntons aggamaglobulinaemia x linked

A

yes

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18
Q

how is bruntons agammaglobulinaemia treated

A

Ig therapy

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19
Q

what sort of infections are patients with b cell defects likely to get

A

Pyogenic bacteria infections, viral infections and infections with strep. pneumonaie

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20
Q

how do pyogenic bacteria infect patients with B cell defects

A

they have polysacccharride capsules which are not recognised by macrophages and neutrophils

21
Q

how are pyogenic bacteria usually cleared

A

complement and antibodies

22
Q

why are people with b cell defects susceptible to viral infections

A

because antibodies are important in neutralising viruses in the gut

23
Q

what virulence factors does streptococcous pneumonaie acquire when it becomes pathogenic when the host is immunocompromised

A

invasins, adhesins and immunogenic cell wall components

24
Q

what sort of immunodeficiency is leaves a patient susceptible to neisseria meningitis

A

complement deficiency

25
Q

what specific defect is linked to susceptibility to infection with niesseria meningitis and why

A

defects in the membrane attack complx as this allows intracellular survival as the host cant perform extracellular lysis

26
Q

what are the two types of innate immunodeficiency

A

defects in inflamatory response and defects in phagocyte killing

27
Q

what is process is effected in chronic granulomatus disease (CGD)

A

phagocyte killing

28
Q

what is the defect in CGD

A

NADPH oxidase

29
Q

what sort of disease is CGD

A

innate immunity

30
Q

how does the defect in CGD result in a compromised immune system

A

it causes difficulty in forming reactive oxygen species which impares bacterial killing

31
Q

what are the results of chronic granulomatus disease

A

recurrant infections incl pneumonia, absesses of the skin, septic arthritis, fungal infection and impetigo

32
Q

what are secondary immunodeficencies which can cause problems with the innate immune system

A

burns, trauma, surgery, smoking,CF, implanted devices, prolonged antibiotic therapy

33
Q

under what circumstances is a person susceptible to infection from staph. aureus and staph. epidermis

A

when the person has defective physical barrier or clearance mechanism

34
Q

what are the results of infection with staph aureus

A

boils or staphylococcal scalded skin syndrome in children

35
Q

what sort of bacteria is Staph. epidermidis

A

gram positive, catalase positive, coagulase negative cocci.

36
Q

which antibiotics is staph epidermidis resistant to

A

vancomycin penicillin and methicillus

37
Q

what is used to treat staph epidermis

A

novabiocin

38
Q

how does staph epidermis form biofilms

A

it produces an extracellular slime which can adhere to plastic surfaces via van der waals and anchor using adhesion molecules such as pili

39
Q

what are common infections in patients suffering from cystic fibrosis

A

s. aureus, haemophilus influenzae, pseudomonas aeruginosa and B. capecea

40
Q

what do the bacteria which commonly infect CF patients have in common

A

they are resistant to common antibiotics

41
Q

what 3 things causesreduced circulating neutrophils

A

radiotherapy, chemotherapy, leukeimia

42
Q

what are the most common infections in patients with phagocyte defects

A

S. aureus, S. pyogenes, Gram negative rods and P. aeruginosa

43
Q

which bacteria are patients with T cell defects likely to be infected by

A

mycobacterium avium and toxoplasma gondii

44
Q

is infection rate of mycobacterium avium inversely related to CD4+ counts or CD8+ counts

A

CD4+

45
Q

what is toxoplasma gondii

A

an intracellular protazoa

46
Q

what percentage of adults does toxoplasma gondii latently infect

A

35%

47
Q

what does the reactivation of latent toxoplasma gondii lead to in T cell deficient patients

A

toxoplasmic encephalitis

48
Q

how does infection from toxoplasma gondii usually occur

A

ingesting oocytes from unwashed veg or tissue cysts in undercooked meat

Immunology (11 decks)

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