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