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Resp JL > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

How many people carry the CF gene?

A

1 in 25

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2
Q

What happens to sweat in CF?

A

Salty

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3
Q

What happens to the pancreas in CF?

A

Fibrotic

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4
Q

What can happen to the Vas Deferens in CF?

A

Congenital bilateral absence

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5
Q

What two diseases are common with CF?

A

Gall bladder and liver disease

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6
Q

What protein is responsible for CF?

A

CFTR protein/ion transport

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7
Q

What does transcription and splicing do to a gene?

A

Removes introns

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8
Q

What organism usually colonises CF patients?

A

Staphlococcus aureus

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9
Q

What can happen to fertility in CF patients?

A

Infertility

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10
Q

What type of bronchiectasis do CF patients get?

A

Upper lobe

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11
Q

What test can confirm CF?

A

Del508

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12
Q

What bacteria colonises the bowels in CF?

A

Pseudomonas

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13
Q

What is given for pseudomonas in CF?

A

Oral azithromycin

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14
Q

What can happen to pancreatic islet cells in endocrine failure in CF?

A

Destroyed, fatty replacement

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15
Q

What is given for CF patients with exocrine failure?

A

CREON

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16
Q

What two drugs are given for pancreatic endocrine failure in CF?

A

Annual OGTT

CGMS

17
Q

What bowel related syndrome can CF patients get?

A

Distal Intestinal Obstruction Syndrome

18
Q

What blocks up the intestine in C?

A

thick mucus

19
Q

What are the three treatments for Distal Intestinal Obstruction syndrome?

A
  1. Gastrograffin
  2. Laxido
  3. Fluids
20
Q

What happens to the hepatic ducts in CF?

A

Sludging up

21
Q

What three things can portal hypertension from CF lead to?

A
  1. Porto-systemic anastomoses
  2. Variceal bleeding
  3. Hepatic encephalopathy
22
Q

What can be done to help portal hypertension in CF?

A

TIPSS

23
Q

What do Tazocin, ceftrazidime, tobramicin, meropenam and colisten all treat?

A

Pseudomonas

24
Q

Give two antibiotics for Staph Aureus?

A

Flucloxacillin

Tigecycline

25
Q

Give an antibiotic for cepacia?

A

Temocillin

26
Q

What mutation is type III, normal CFTR, non-functioning channel and is called the celtic gene?

A

G551D mutation

27
Q

Name the drug that is a CFTR potentiator?

A

Ivacaftor

28
Q

What are the two CF mutations in exons?

A
  1. F508Del (deletion of phenylalanine position 508)

2. G551D (glycine to asparagine at amino acid 551)

Resp JL (27 decks)

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