[cystic fibrosis] Flashcards
AR
caucasian
CFTR (CF transmembrane conductance regulator)
chloride (reduced reabsorption of Cl- from sweat into sweat duct, so increased Cl-. This ‘traps’ more Na in the sweat as well)
bronchiectasis
chronic infection
Sweat test
NaCl are >60 mmol/L
haemoptysis
wheeze
cough
pancreatic insufficiency (DM + steatorrhoea)
osteoporosis
sinusitis
vasculitis
hypertrophic pulmonary osteoarthropathy
clubbing of distal phalanges
periostitis (periosteum inflammation)
cirrhosis
obstructive
obstructive
Postural drainage
Abx or acute infective exacerbations
pancreatic enzyme replacement
ursodeoxycholic acid (liver replacement)
ADEK (fat soluble)
bilateral coarse crackles
faecal elastase
chromosome 7
bronchiectasis
pneumothorax
thickened secretions thickened secretions by liver to aid with digestion blocks ducts cirrhosis clotting factors etc
thickened secretions thickened secretions by liver to aid with digestion blocks ducts cirrhosis clotting factors affected
thickened bile secretions block exocrine function results in chronic damage to pancreas painful pancreatitis DM/steatorrhoea/ADEK
Staph Aureus
Haemophilus influenzae
pseudomonas aeruginosa