Cystic fibrosis COPY Flashcards

1
Q

Ivacaftor

A

-CFTR potentiator: increase CFTR activity (targets class 3 mutations), binds to CFTR channel to increase probability of gate opening

  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
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2
Q

Lumacaftor

A
  • Acts a chaperone to improve CFTR folding
  • CFTR corrector: Correct folding and trafficking (class 2 mutations)
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
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3
Q

Tezacaftor

A
  • Alters CFTR protein to improve trafficking to cell surface
  • CFTR corrector: correct folding & trafficking
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
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4
Q

Elexacaftor

A
  • Alters CFTR protein to improve trafficking to cell surface
  • CFTR corrector: correct folding & trafficking
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
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5
Q

Hypertonic Saline & Mannitol

A

Airway hydrators

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6
Q

Mucolytics

A

Inhaled n- acetylcysteine: cleaves disulfide linkages in mucus

Inhaled dornase alfa: DNAse endonuclease that cleaves long sticky DNA polymers in mucus to reduce sputum viscosity & elasticity

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7
Q

-CFTR potentiator: increase CFTR activity (targets class 3 mutations), binds to CFTR channel to increase probability of gate opening

  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
A

Ivacaftor

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8
Q
  • Acts a chaperone to improve CFTR folding
  • CFTR corrector: Correct folding and trafficking (class 2 mutations)
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
A

Lumacaftor

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9
Q
  • Alters CFTR protein to improve trafficking to cell surface
  • CFTR corrector: correct folding & trafficking
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
A

Tezacaftor

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10
Q
  • Alters CFTR protein to improve trafficking to cell surface
  • CFTR corrector: correct folding & trafficking
  • CFTR Modulator: Improve airway surface liquid layer
  • Genetic testing is always required since they’re designed for specific mutations
A

Elexacaftor

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11
Q

Airway hydrators

A

Hypertonic Saline & Mannitol

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12
Q

Inhaled n- acetylcysteine: cleaves disulfide linkages in mucus

Inhaled dornase alfa: DNAse endonuclease that cleaves long sticky DNA polymers in mucus to reduce sputum viscosity & elasticity

A

Mucolytics

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