transports chloride across apical membrane, regulates fluid/electrolyte balance in mucus membranes, secretes bicarbonate
What are the CFTR ion channel's functions? (3)
F508 missense
What type of mutation is involved in CF?
abnormal trafficking; protein gets degraded; no functional protein
What happens to the ion channel in CF? (3)
depleted water content, viscous mucus, obstruction/inflammation of airways, infection, remodeling, respiratory failure
What is the pathophysiological cascade in CF? (6)
pancreatic enzyme RT, vitamin/mineral/electrolyte RT, choleretic therapy
What are the treatments for GI & hepatic care? (3)
b
Which is given prophylactically to increase bile secretions from the liver?
a) Vitamin/Mineral/Electrolyte RT
b) Choleretic Therapy
c) Pancreatic Enzyme RT
c
Which is given to gain & maintain a healthy weight?
a) Vitamin/Mineral/Electrolyte RT
b) Choleretic Therapy
c) Pancreatic Enzyme RT
amylase, lipase, protease
Which enzymes are given? (3)
lung transplant
What is the only treatment option available for End-stage lung disease?
a
Which hydrates the airway's mucus secretion & facilitates mucociliary
function?
a) Hypertonic saline
b) Albuterol
c) Pulmozyme
d) Levalbuterol
b,d
What are used to relieve airway obstruction?
a) Hypertonic saline
b) Albuterol
c) Pulmozyme
d) Levalbuterol
recombinant dornase alpha
Pulmozyme generic name?
DNase that cleaves neutrophilic network and decreases the viscosity of mucus
Pulmozyme MOA?
pseudomonas, staph. aureus, h.flu
What are the bacteria involved in acute exacerbations? (3)
break down biofilm produced by pseudomonas
Why do we give azithromycin?
lumacaftor, tezacaftor, elexacaftor
What are the correctors/chaperones? (3)
ivacaftor
What is the potentiator therapy?
kalydeco
Ivacaftor Brand name?
ivacaftor + lumacaftor
Orkambi=
ivacaftor + tezacaftor
Symdeko=
ivacaftor + tezacaftor + elexacaftor
Trikafta =
b
Which has the most AE's & is less effective?
a) tezacaftor
b) lumacaftor
c) elexacaftor
d) ivacaftor
c
Which was developed for triple combo therapy?
a) tezacaftor
b) lumacaftor
c) elexacaftor
d) ivacaftor
a
Which mutation results in no protein?
a) Class I
b) Class II
c) Class III
d) Class IV
b
Which mutation results in No traffic?
a) Class I
b) Class II
c) Class III
d) Class IV
c,d
Which mutation results in no function or less function?
a) Class I
b) Class II
c) Class III
d) Class IV
class III; potentiator (ivacaftor)
Which class is the no-function mutation? What therapy do they need?