15. Cystic Fibrosis Flashcards
genetic mutation of cystic fibrosis?
chromosome?
gene?
inheritance pattern?
3 nucleotide deletion (508)
7
CFTR
AR
3 major organs affected in CF?
lung
pancreas
small intestine
85% of CF pts present w what?
15%
pancreatic insufficiency
intestinal blockage (meconium ileus)
diagnostic test for CF?
sweat salt test
most common organism causing pneumonia in kids w CF?
not in kids?
S. aureus
P. auriginosa
structure of pseudomonas auriginosa?
gram negative
motile
rod
3 clinical manifestations of CF?
localized lung infections in bronchi and bronchioles
neutrophil infiltration
malnutrition
2 virulence factors in pseudomonas?
alginate productions –> mucoidy
bacterial biofilms
character of nonmucoid alginate?
mucoid?
nonmucoid - smooth LPS, high proteases
mucoid - rough LPS, low proteases
wt happens in primary TB? 6
bact inhaled bact enter macrophages and multiply lesion forms lesion liquefies spreads to blood and other organs death
wt form of TB is found in the apex?
secondary
phenotype of alginate producing pseudomonas?
mucoidy
4 things causing lung tissue destruction in chronic infeciton associated with CF?
IC- mediated
inflammation
type 3 hypersensitivity
chronic infectoin
tx for CF?
supresive therapy for inflammaiton
inhaled tobramycin and oral azithromycin
3 things mycobacterium is resistanct to?
drying
chemicals
germicides
what allows MTB to escape phagocytic vesicles?
LLO homologue (hemolysin)
how does MTB prevent acidification in phagosome?
producing NH4
what causes lung damage in TB?
TNF-a released from macrophages in response to cell wall components
wt cells are found in caseous granuloma of TB?
central core offused macrophages
epitheloid cell
CD4 + T cells
complex seen in TB? wtype of cells?
Ghon complex
Langhan’s cells
wt is in the tuberculin skin test?
pts with false negative?
pts w false positives?
Purified protein derivative from mycobacterium cell wall
- in AIDS
+ in other mycobacteria sp. and BCG vaccine
hw long to treat TB?
6 months
is response to biofilm chronic or acute?
chronic
wt allows pseudomonas resistance to antibiotics?
biofilm
wt is an epitheloid cell?
activated macrophages resembling epithelial cells
wt is the role of Th1 cells in TB?
release IFN to activate macrophages
