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Conditions: Respiratory > CF > Flashcards

CF Flashcards

1
Q

What is the pattern of inheritence of CF?

A
  • Autosomal recessive
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2
Q

How many people are affected in N. Europe?

A
  • 1 in 25
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3
Q

What gene is responsible?

A
  • C7q
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4
Q

What does C7q code for?

A
  • Cystic fibrosis transmembrane conductance regulator (CFTR) (a chloride channel)
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5
Q

What is the most common CF resulting mutation?

A
  • Phe508del
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6
Q

What is the function of CFTR?

A
  • Active transport for chloride

- Regulates liquid volume on epithelial surfaces

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7
Q

What does the abnormality of CFTR lead to?

A
  • Decreased mucociliary clearence
  • Increase bacterial adherence
  • Reduced endocytosis of bacteria
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8
Q

What do the consequences of abnormal CFTR lead to?

A
  • Bacterial colonisation
  • Inflammation
  • Mucous plugging
  • Airway damage and ulceration
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9
Q

What airway disease can CF commonly lead to?

A
  • Bronchiectasis
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10
Q

Why is CF a multisystem disease?

A
  • Chloride movement occurs all over the body
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11
Q

What does CF cause with Na?

A
  • Causes Na retention too due to the tight association of Na+ and Cl-
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12
Q

What two features are common in 80% of CF sufferers?

A
  • Recurrent pulmonary infections

- Pancreatic insufficiency

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13
Q

What other features to other systems of CF are there?

A
  • Pulmonary infection on it’s own

- GI problems

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14
Q

What can result from the recurrent chest infections of CF and can be seen on examination?

A
  • Pneumonitis
  • Bronchiectasis
  • Scarring
  • Abscesses
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15
Q

Why do people with CF have chronic purulent sputum production?

A

Due to the bronchiectasis

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16
Q

What do the pancreatic issues commonly result in?

A

Onset diabetes

17
Q

What other systemic signs are evident in CF?

A
  • Weight loss

- Pyrexia

18
Q

What are the pulmonary signs on examination of CF?

A
  • Haemoptysis
  • Pneumothorax
  • Nasal polyps
  • Stony dull percussion
  • Crepitations
  • Increased vocal resonance
  • Bronchial breathing in vesicular areas
19
Q

What would cause the haemoptysis in CF?

20
Q

Who is more at risk of a CF associated pneumothorax?

A
  • Older males
21
Q

What causes the abnormal stools in CF?

A
  • Pancreatic insufficiency
22
Q

What tests are performed to diagnose CF?

A
  • Guthrie (heel prick test) on newborns
23
Q

What would be shown on the guthrie test to diagnose CF?

A
  • Immunoreactive trypsinogen
24
Q

If positive for immunoreactive trypsinogen what tests are performed?

A
  • Mutation analysis tests

- Sweet chloride test

25
What can be done to analyse need for a lung transplant?
- Pulmonary function test/spirometry
26
If infected with pseudemonas aeruginosa, what should be course of treatment?
- Oral ciprofloxacin - Nebulised colomycin - If that failis IV cefrazidime
27
What types and combinations of antibiotics should be used to treat infections in individuals with CF?
2 antibiotics - B lactams - Aminoglycosides
28
What should be given in tandem with the antibiotics?
- Anti inflammatories to reduce inflammation in the airways
29
What if ivacaftor?
- New class of drug addressing primary defect if CFTR gene - Improves Cl transport - Only works in 5-10% of patients with a certain mutation (G551D)
30
How do you treat the associated pancreatic insufficiency of CF?
- Enteric coated enzyme pellets - High energy diet (not low fat) - Fat soluble vitamin and vitamin supplements - PP inhibitors
31
Name 6 bacteria that commonly infect CF sufferers in order of likelihood
- Pseudomonas Aeruginosa - Staph A - H. Influenzae - Stenothrophomanas Maltaphilia - Burkholderia Cepacia - Mycobacterium abscessus
32
How are CF sufferers infected with pseudemonas aeruginosa?
- Environment - HAI - Other CF sufferers - Lack of hygiene
33
What happens after pseudomonas aeruginosa infects a CF patient?
- Undergoes mucoid change | - Forms biofilm to protect from host defences
34
What does the formation of the biofilm speed up?
- Antibiotic resistance
35
Where is burkholderia cepacia found?
Onion rot
36
What is colonisation of burkholderia cepacia associated with
- RAPID DECLINE IN LUNG FUNCTION | - SIGNIFICANTLY SHORTENED LIFE EXPECTANCY
37
How many of the pathogens common in CF are resistant to many antibiotics?
- Pseudomanas gains it quickly - Burkholderia resistant - Srenothrophomonas resistant - Mycobacterium resistant to ALL anti-TB chemotherapy
38
What antibiotic is used for mycobacterium abscessus?
- Azithromycin
39
How does CF affect male fertility?
- Blocked or absent vas deferens

Conditions: Respiratory (16 decks)

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