15. Cystic Fibrosis

Question 1

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

Answer 1

3 nucleotide deletion (508)
7
CFTR
AR

Question 2

3 major organs affected in CF?

Answer 2

lung
pancreas
small intestine

Question 3

85% of CF pts present w what?

15%

Answer 3

pancreatic insufficiency

intestinal blockage (meconium ileus)

Question 4

diagnostic test for CF?

Answer 4

sweat salt test

Question 5

most common organism causing pneumonia in kids w CF?

not in kids?

Answer 5

S. aureus

P. auriginosa

Question 6

structure of pseudomonas auriginosa?

Answer 6

gram negative
motile
rod

Question 7

3 clinical manifestations of CF?

Answer 7

localized lung infections in bronchi and bronchioles
neutrophil infiltration
malnutrition

Question 8

2 virulence factors in pseudomonas?

Answer 8

alginate productions –> mucoidy

bacterial biofilms

Question 9

character of nonmucoid alginate?

mucoid?

Answer 9

nonmucoid - smooth LPS, high proteases

mucoid - rough LPS, low proteases

Question 10

wt happens in primary TB? 6

Answer 10

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

Question 11

wt form of TB is found in the apex?

Answer 11

secondary

Question 12

phenotype of alginate producing pseudomonas?

Answer 12

mucoidy

Question 13

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

Answer 13

IC- mediated
inflammation
type 3 hypersensitivity
chronic infectoin

Question 14

tx for CF?

Answer 14

supresive therapy for inflammaiton

inhaled tobramycin and oral azithromycin

Question 15

3 things mycobacterium is resistanct to?

Answer 15

drying
chemicals
germicides

Question 16

what allows MTB to escape phagocytic vesicles?

Answer 16

LLO homologue (hemolysin)

Question 17

how does MTB prevent acidification in phagosome?

Answer 17

producing NH4

Question 18

what causes lung damage in TB?

Answer 18

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

Question 19

wt cells are found in caseous granuloma of TB?

Answer 19

central core offused macrophages
epitheloid cell
CD4 + T cells

Question 20

complex seen in TB? wtype of cells?

Answer 20

Ghon complex

Langhan’s cells

Question 21

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

Answer 21

Purified protein derivative from mycobacterium cell wall

• in AIDS

+ in other mycobacteria sp. and BCG vaccine

Question 22

hw long to treat TB?

Answer 22

6 months

Question 23

is response to biofilm chronic or acute?

Answer 23

chronic

Question 24

wt allows pseudomonas resistance to antibiotics?

Answer 24

biofilm

Question 25

wt is an epitheloid cell?

Answer 25

activated macrophages resembling epithelial cells

Question 26

wt is the role of Th1 cells in TB?

Answer 26

release IFN to activate macrophages