Cystic fibrosis, pneumonia

Question 1

Cystic fibrosis

Answer 1

1. AR, CFTR - chromosome 7
2. epithelial cells cannot secrete Chloride ion = thick secretions

lungs - infection - pneumonia - mucopurulent cough - bronchiectasis Pancreas - malabsorption - thick bulky fatty stools

Failure to thrive
Rectal prolapse

Question 2

CF DXT

Answer 2

BEST : SWEAT test

other:
1. Pancreatic function - 72hr fecal fat collection
2. Inc trypsinogen - newborn
3. Stool trypsin
4. DNA test

No rx
Symptomatic mx

Question 3

Pneumonia in children

Answer 3

Community acq

Uncomplicated
1. CXR NOT REQ
2. Amoxicillin oraly
-if not tolerated
IV benzyl penicillin

Complicated:
parapneumonic effusion sev resp distress
` marked tachy
O2 sats <80% Altered mental state
1. IV ceftriaxone + IV Flucloxacin

Question 4

When to do CXR in pneumonia

Answer 4

1. Not in uncomplicated CAP
2. In complicated cases
3. No improvement after 2-3days of antibiotic therapy

F/U cxr after 6wks
1. Complicated pneumonia
2. Recurrent

Question 5

Parapneumoni