CF + AAT

Question 1

which bacteria colonise in CF

Answer 1

Staph aureus
H.Influenza
Pseudoamonas
Asp. fumigatus
Burk. cepacia

Question 2

what is the inheritance of CF

Answer 2

1/ 2500 live births
1/20 carrier status
A Recessive
chloride channel fault
delta f508 CFTR on Ch7.

Question 3

what is treatment of CF

Answer 3

daily chest PT
Nebulised DNA
IV abx
heart / lung transplant

Question 4

features of CF:

Answer 4

muconeum ileus
pneumothorax due to rupture of bullae

rectal prolapse
panc. insufficiency
subfertiility

Question 5

what are the genetics for a1 antitrypsin deficiency?

Answer 5

ch. 14
A Recessive

M = norMal
S = Slow (80% of normal)
Z = very slow (15% of normal)

Question 6

what are the features of a1-antitrypsin deficiency?

Answer 6

emphysema
liver cirrhosis

Question 7

how is a1-antitrypsin deficiency diagnosed?

Answer 7

protease inhibitor activity

Question 8

What is the role of A1 Anti-Trypsin?

Answer 8

• made in the liver
• to protect lungs

protease inhibitor inhibits neutrophil elastase activity causing destruction