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Flashcards in Cystic Fibrosis Deck (14):

What regulator is wrong in CF?

The trans-membrane conductance regulator that channels out Cl. Slide 4


What are consequences of CF?

Salty sweat
Fibrotic pancreas
Failure to thrive
Recurrent baterial lung infections
Gallbladder and Liver Disease
Absence of Vas Deferens. Slide 5


How many classes of defects in the CFTR gene are there and what are they?

Class I: Don't survive
Class II: Little activity as protein is wrong, severe CF
Class III: Doesn't open
Class IV: Doesn't open properly
Class V: Not enough
Slide 6


What are the 2 different mutations in the CFTR gene and which is the most common?

F508del and G551D.
The most common is the F508del. Slide 20


Why can come patients present with CF as adults and not been diagnosed at a younger age?

Don't present
Present out of hours and means there is lack of consistent record
Failure to think about it
Slide 26


In CF what goes wrong with the pancreas?

Exocrine - no secretion of lipases and digestive failure
Endocrine failure -
Islet cells fail so diabetes develops.
Slide 42


In CF what goes wrong with the bowels?

Thick mucus blocks S and L intestine and symptoms like constipation.
Slide 43


How do you treat and prevent bowel problems in CF patients?

Treatment: Grastrograffin, laxido and fluids
Prevention: Laxido, hydration and keep moving promotes bowel movement. Slide 43


In CF what goes wrong with the liver?

Sludging up of the hepatic ducts
Portal hypertension. Slide 44


If a CF patient had staph aureus what ABX would they be on?

Flucloxacillin and tigecycline. Slide 46


What is OHPAT?

Outpatient and Home Parenteral Antibiotic Treatment
Allows antibiotics to be self administered.
Slide 47


What type of class does someone have if they have the G551D mutation?

Type III mutation so they have non functioning channels.
Slide 50


What is Ivacaftor?

They CFTR potentiator that improves Cl flow and is specifically for the G551D mutation, however is very expensive. Slide 51


What is the drug that targets the F508del mutation in CF?

Lumacaftor. Slide 58

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