Cystic Fibrosis Flashcards
1
Q
Cystic Fibrosis
A
Autosomal recessive
Abnormal chloride channels (CFTR) in lumInal surface lead to increased reabsorption of Na and H20 leading to increased viscosity of airway secretions
2
Q
Complications
A
Congenital cause of bronchiectasis Tenacious sputum Biliary obstruction Pancreatic dysfunction Infertility for males
3
Q
Signs
A
Recurrent chest infections in childhood, failure to thrive, bronchiectasis
4
Q
Symptoms
A
Clubbing
Purulent sputum
Central cyanosis
Bilateral coarse crackles
5
Q
Testing
A
Sweat test
Del508 genetic test
Immunoreactive trypsin assay
6
Q
Management
A
Antibiotics Postural drainage Pancreatic supplements Low fat diet Bronchodilators Heart-lung transplant
7
Q
Epidemiology
A
1/25 carry gene
8
Q
Bronchiectasis <40 years
A
screen for CF
9
Q
Genetic causes
A
Del508
G551D, causes nonfunctioning channel
10
Q
Ivacaftor
A
Could be solution
Improves CFTR flow
EXPENSIVE
11
Q
Exacerbation management
A
Antibiotics
Physiotherapy
Hydration
Increased dietary input
12
Q
OHPAT
A
Outpatient home parenteral antibiotic therapy
