Cystic Fibrosis Flashcards

1
Q

What is the primary cause of cystic fibrosis?

A

A defect in the gene that codes for the cystic fibrosis trans-membrane conductance regulator

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2
Q

How does the CFTR cholrine channel normally function?

A

The CFTR channel pumps cholride ions out of epithelial cells

The activation of the channel allows the functioning of surrounding chloride channels

The sodium transporter is inhibited meaning sodium cannot enter cells

This means osmosis will be directed outwards into the airways keeping them moist

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3
Q

How does a CFTR mutation cause cystic fibrosis in the airways?

A

Cholrine cannot be transported across the cell membrane via the CFR channel or the surrounding cholide channels which canow now not be activated

Inhibition of the sodium ion transporter ceases

Sodium enters the cell reversing the osmotic gradient into the cell

This dries out the aiwards creating very thick sputum

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4
Q

What is the most common mutation in cystic fibrosis?

A

Deletion of phenylalanine at position 508

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5
Q

What is a promising therapy for cystic fibrosis?

A

Lumacaftor

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6
Q

How is cystic fibrosis treated if infected with staph aureus?

A

Oral flucloxacillin

Oral septrin

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7
Q

How is cystic fibrosis treated if infected with pseudomonas?

A
  • Oral azithromycin
  • Nebulised colomycin
  • Nebulised or inhaled tobramycin
  • Nebulised aztreonam
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8
Q

Cystic fibrosis affects the lungs, but which other parts of the body are affected?

A
  • Pancreas
  • Bowels
  • Liver
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9
Q

How does cystic fibrosis affect the exocrine function of the pancreas?

A

Pancreatic ducts are blocked and lipases and amylase cannot be secreted

Digestive failure occurs

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10
Q

How can the exocrine functioning of the liver be treated when cystic fibrosis is present?

A

CREON

(pancrelipase)

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11
Q

How does cystic fibrosis affect pancreatic endocrine function?

A

Pancreatic islet cells are destroyed and replaced with fatty tissue

This can cause diabetes and insulin needs to be administered

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12
Q

How can the bowels be affected by cystic fibrosis?

A

Distal intestinal obstruction syndrome

Mucus blocks the small and large intestines

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13
Q

How is distal intestinal obstruction syndrome (DIOS) treated?

A

Additional fluids

Gastrografin - a contrast medium to locate areas of blockage

Laxido (laxiative)

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14
Q

How can the liver be affected by cystic fibrosis?

A

Hepatic ducts become blocked

This can lead to portal hypertension causing variceal bleeding

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15
Q

What is hepatic encephalopathy?

A

A decrease in brain function due to brain damage by a build up in toxins the liver fails to remove

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16
Q

Why should antibiotics always be used in pairs?

A

Reduced instance of resistance

17
Q
A