Cystic fibrosis Flashcards
1
Q
What is pathophysiology of cystic fibrosis?
A
Autosomal recessive disorder which affects the CTR Gene.
In new born it causes thick sticky stools called meconium + this can get stuck in intestines known as meconium ileus.
In childhood it can block + damage pancreas + cause fibrosis.
Later in childhood it can cause lung problems as cillia wont be able to move mucus as its very thick meaning bacteria in the mucus can collonize
2
Q
Complications of cystic fibrosis?
A
Bronchiectasis
Hemoptysis
Respiratory failure
Infertility
Digital clubbing of nails
3
Q
Diagnosis of cystic fibrosis?
A
Heel prick test for new born:
- show pancreatic enzyme IRT
Sweat test for older
- Detect increase chloride