Cystic Fibrosis Flashcards
How many people carry the CF gene?
1 in 25
What happens to sweat in CF?
Salty
What happens to the pancreas in CF?
Fibrotic
What can happen to the Vas Deferens in CF?
Congenital bilateral absence
What two diseases are common with CF?
Gall bladder and liver disease
What protein is responsible for CF?
CFTR protein/ion transport
What does transcription and splicing do to a gene?
Removes introns
What organism usually colonises CF patients?
Staphlococcus aureus
What can happen to fertility in CF patients?
Infertility
What type of bronchiectasis do CF patients get?
Upper lobe
What test can confirm CF?
Del508
What bacteria colonises the bowels in CF?
Pseudomonas
What is given for pseudomonas in CF?
Oral azithromycin
What can happen to pancreatic islet cells in endocrine failure in CF?
Destroyed, fatty replacement
What is given for CF patients with exocrine failure?
CREON
What two drugs are given for pancreatic endocrine failure in CF?
Annual OGTT
CGMS
What bowel related syndrome can CF patients get?
Distal Intestinal Obstruction Syndrome
What blocks up the intestine in C?
thick mucus
What are the three treatments for Distal Intestinal Obstruction syndrome?
- Gastrograffin
- Laxido
- Fluids
What happens to the hepatic ducts in CF?
Sludging up
What three things can portal hypertension from CF lead to?
- Porto-systemic anastomoses
- Variceal bleeding
- Hepatic encephalopathy
What can be done to help portal hypertension in CF?
TIPSS
What do Tazocin, ceftrazidime, tobramicin, meropenam and colisten all treat?
Pseudomonas
Give two antibiotics for Staph Aureus?
Flucloxacillin
Tigecycline
Give an antibiotic for cepacia?
Temocillin
What mutation is type III, normal CFTR, non-functioning channel and is called the celtic gene?
G551D mutation
Name the drug that is a CFTR potentiator?
Ivacaftor
What are the two CF mutations in exons?
- F508Del (deletion of phenylalanine position 508)
2. G551D (glycine to asparagine at amino acid 551)
