Cystic Fibrosis Flashcards Preview

1. Respiratory > Cystic Fibrosis > Flashcards

Flashcards in Cystic Fibrosis Deck (7)
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1
Q

What is the most common mutation causing CF?

A

F508del

2
Q

What causes CF?

A

Defects in the CFTR (cystic fibrosis transmembrane regulator)

3
Q

What are the consequences of CF?

A
Bronchiectasis – Cystic and saccular 
Tenacious sputum 
Biliary obstruction, and obstructive hepatitis 
Pancreatic dysfunction 
– Endocrine – CFRDM
– Exocrine - steatorrhoea 
Infertility for males

Psychological issues for all
4
Q

When would you investigate a case for CF?

A
Bronchiectasis under 40
Upper lobe bronchiectasis
Colonisation with Staph
Infertility
Low weight
5
Q

How do you manage CF?

A
Antibiotics
•Staph Aureus Colonised
–Oral Fluclox
–Oral Septrin
•Pseudomonas Colonised
–Oral Azithromycin
–Nebulised Colomycin
–Nebulised Tobramycin
6
Q

What drug do you take for the F508del mutation?

A

Lumacaftor drug

7
Q

Describe the G551D gene?

A
The Celtic Gene
Type III Mutation
Normal CFTR
Delivered to the epithelium normally
Non-functioning channel