Cystic Fibrosis Flashcards

1
Q

What is CF?

A

A disease affecting epithelial cells
Changes iob transport across epithelial cell membrane
Affects movement of chlorine
Changes viscosity and tenacity of mucus

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2
Q

What does CF mainly affect?

A

Lungs, Pancreas, Sweat Glands, Ductus defferents

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3
Q

Genetic

A

Autosomal recessive
1:25 people carriers, 1:22 caucasians, 1:2500
Triple base deletion on chromosome 7CFTR gene rendundant, responsible for transmembrane Cl- transport

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4
Q

Symptoms

A

Thich mucus in lungs hard to clear, causing infection
Pancreas
Poor pancreatic secretion
Less pancreatic lipase, fats not absorbed
Steattorhea
Sweat - high sodium, salty
Ductus defferens - Not formed in 90% of CF cases

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5
Q

How is CF managed?

A

Limit lung infections Improve ion transport

Deliver normal CFTR gene

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