Cystic fibrosis

Question 1

Consequences of CF

Answer 1

Salty sweat 
Intestinal blockage 
Fibrotic pancreas
Failure to thrive 
Recurrent bacterial infections 
Absence of vas deferens 
Filled sinuses 
Gallbladder & liver disease

Question 2

Respiratory symptoms of CF

Answer 2

Frequent chest infections 
Sinusitis 
Nasal polyps 
Bronchiectasis 
Spontaneous pneumothorax 
Respiratory failure 
Cor pulmonae

Question 3

Respiratory signs of CF

Answer 3

Cyanosis
Finger clubbing
Bilateral coarse crackles

Question 4

GI symptoms/signs of CF

Answer 4

Steatorrhoea
Meconium ileus
Cholesterol gallstones 
Cirrhosis 
Peptic ulcerations

Question 5

Other general symptoms/signs of CF

Answer 5

Malnutrition 
Pulmonary sepsis 
Failure to thrive 
Females are infertile 
Makes have no vas deferens (infertile) 
Diabetes melitjs 
Arthropathy

Question 6

Investigations for CF

Answer 6

Family history (genetic screening) 
Sweat ( Na conc > 60mmol/L, chloride > sodium)
Blood DNA analysis 
Absent vas deferens & epididymis 
Tests for common presentations

Question 7

General care for CF patients

Answer 7

Stop smoking
Flu & pneumococcal vaccines
Pulmonary rehab

Question 8

Treatment of CF

Answer 8

Oxygen therapy
Antibiotics (nebulised for resp infection)
Drug therapy (B2 agonist & corticosteroids)
Airway clearance (DNase 2.5mg daily improves FEV)
Pancreatic enzyme replacement (creon)
Treat other diseases caused by CF

Question 9

What is CF?

Answer 9

Autosomal recessive condition
Mutation in CFTR gene on chromosome 7
Problem with chloride channel in lumen (absent or doesn’t work)
Channel doesn’t open in response to increased cAMP
Increased Na reabsorption
Less, salt excretion, less water excretion, airway mucous thickens