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

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2
Q

Complications of cystic fibrosis?

A

Bronchiectasis
Hemoptysis
Respiratory failure
Infertility
Digital clubbing of nails

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3
Q

Diagnosis of cystic fibrosis?

A

Heel prick test for new born:
- show pancreatic enzyme IRT

Sweat test for older
- Detect increase chloride

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