15. Cystic Fibrosis Flashcards Preview

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Flashcards in 15. Cystic Fibrosis Deck (26)
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1
Q

genetic mutation of cystic fibrosis?
chromosome?
gene?
inheritance pattern?

A

3 nucleotide deletion (508)
7
CFTR
AR

2
Q

3 major organs affected in CF?

A

lung
pancreas
small intestine

3
Q

85% of CF pts present w what?

15%

A

pancreatic insufficiency

intestinal blockage (meconium ileus)

4
Q

diagnostic test for CF?

A

sweat salt test

5
Q

most common organism causing pneumonia in kids w CF?

not in kids?

A

S. aureus

P. auriginosa

6
Q

structure of pseudomonas auriginosa?

A

gram negative
motile
rod

7
Q

3 clinical manifestations of CF?

A

localized lung infections in bronchi and bronchioles
neutrophil infiltration
malnutrition

8
Q

2 virulence factors in pseudomonas?

A

alginate productions –> mucoidy

bacterial biofilms

9
Q

character of nonmucoid alginate?

mucoid?

A

nonmucoid - smooth LPS, high proteases

mucoid - rough LPS, low proteases

10
Q

wt happens in primary TB? 6

A
bact inhaled
bact enter macrophages and multiply
lesion forms
lesion liquefies
spreads to blood and other organs
death
11
Q

wt form of TB is found in the apex?

A

secondary

12
Q

phenotype of alginate producing pseudomonas?

A

mucoidy

13
Q

4 things causing lung tissue destruction in chronic infeciton associated with CF?

A

IC- mediated
inflammation
type 3 hypersensitivity
chronic infectoin

14
Q

tx for CF?

A

supresive therapy for inflammaiton

inhaled tobramycin and oral azithromycin

15
Q

3 things mycobacterium is resistanct to?

A

drying
chemicals
germicides

16
Q

what allows MTB to escape phagocytic vesicles?

A

LLO homologue (hemolysin)

17
Q

how does MTB prevent acidification in phagosome?

A

producing NH4

18
Q

what causes lung damage in TB?

A

TNF-a released from macrophages in response to cell wall components

19
Q

wt cells are found in caseous granuloma of TB?

A

central core offused macrophages
epitheloid cell
CD4 + T cells

20
Q

complex seen in TB? wtype of cells?

A

Ghon complex

Langhan’s cells

21
Q

wt is in the tuberculin skin test?
pts with false negative?
pts w false positives?

A

Purified protein derivative from mycobacterium cell wall

  • in AIDS

+ in other mycobacteria sp. and BCG vaccine

22
Q

hw long to treat TB?

A

6 months

23
Q

is response to biofilm chronic or acute?

A

chronic

24
Q

wt allows pseudomonas resistance to antibiotics?

A

biofilm

25
Q

wt is an epitheloid cell?

A

activated macrophages resembling epithelial cells

26
Q

wt is the role of Th1 cells in TB?

A

release IFN to activate macrophages