Cystic Fibrosis: Adults Flashcards

1
Q

What percentage of patients have recurrent pulmonary infections and pancreatic insufficiency?

A

80%

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2
Q

Why is there persistent respiratory infections in CF?

A

Decrease in mucociliary clearance

Increase in bacterial adherence

Reduce in endocytosis of bacteria

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3
Q

What is the effect of CF on bronchiectasis?

A

Causes Aggressive progressive bronchiectasis: chronic sputum production (purulent

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4
Q

What is the result of decreased mucociliary clearance?

A

Increased bacterial colonisation

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5
Q

What is the result of increased bacterial colonisation?

A

Inflammation

Mucus plugging

Airway ulceration

Airway damage

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6
Q

What is the result of Inflammation

Airway plugging

Airway ulceration

Airway Damage?

A

Bronchiectasis?

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7
Q

What is the result of bronchiectasis?

A

More

Decreased mucociliary clearance

Increased bacterial adherence

Decreased endocytosis

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8
Q

Describe the onset of bronchiectasis in CF patients?

A

Aggressive and progressive - chronic sputum production

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9
Q

What respiratory problems besides bronchiectasis do CF patients face?

A

Recurrent lower respiratory tract infections

Progressive airflow obstruction (survival rate related to FEV1)

Increase in exertional dyspnoea

Haemoptysis (associated with infection - possible embolisation)

Pneumothorax- poor prognosis

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10
Q

Describe the onset of colonisation of pseudomonas aeruginosa

A

21% colonisation under 1 year

80% colonisation over 26 years

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11
Q

Where is pseudomonas aeruginosa acquired from?

A

Environment (particularly hospitals)

Other CF patients

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12
Q

How does pseudomonas aeruginosa protect itself from host defences?

A

Once colonised, it undergoes mucoid change. It forms a biofilm - rapidly acquires antibiotic resistance

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13
Q

What is colonisation of pseudomonas aeruginosa associated with?

A

Reduced life expectancy 28 vs 39 years

Rapid decline in lung function

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14
Q

How does the body respond to the presence of a biofilm?

A

Neutrophils and macrophages release proteinases and oxidants (free radicals) produce tissue damage

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15
Q

What is treatment for first isolation of pseudomonas Aeruginosa?

A

Attempt eradication with oral ciprofloxacin & nebulised colomycin 3/12

If fails, i.v. ceftazidime & nebulised colomycin

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16
Q

Where is Burkholderia cepacia acquired from?

A

Environment

Other CF patients

17
Q

What is Colonisation of Burkholderia cepacia associated with?

A

Reduced life expectancy 16 vs 39 years

Rapid decline in lung function

Some patients ‘cepacia syndrome,’ very rapid deterioration

18
Q

What is the resistance of burkholderia cepacia?

A

Innate resistance to most antibiotics

19
Q

What does Genomovar 3 indicate (a group of Burkholderia cepacia-like organisms)?

A

Contraindication for transplantations

20
Q

Which organism is usually secondary to pseudomonas?

A

Stentrophomonas maltophila

21
Q

Which bacterium is resistant to all anti-tuberculosis chemotherapy and is a contraindication for transplantation.

A

Mycobacterium abscessus

22
Q

How do you treat recurrent respiratory tract infections?

A

Early and aggressively with antibiotics

23
Q

What bacteria would you treat with oral antibiotics?

A

Staph, Haemophilus, Pneumococcus

24
Q

Which bacteria do you treat with IV antibiotics?

A

Pseudomonas, Stenotrophomonas, Burkholderia

25
Q

What is the format of antibiotic administration?

A

Two antibiotics (b-lactam + aminoglycoside)

If multiply resistant, test for synergy between antibiotic combinations

Large doses (increased volume of distribution, increased clearance)

Two week courses

26
Q

What is the function of the Ivacaftor?

A

Addresses primary defect of CTFR

CTFR potentiator, binds to CTFR and improves the transport of chloride ions

Improves lung function (10%)

Weight gain

Reduces sweat chloride

Feel much better

27
Q

What are the indications for a double lung transplant?

A

Rapidly deteriorating lung function

FEV1 < 30% predicted

Life threatening exacerbations

Estimated survival <2 years

28
Q

What is the likelihood of dying on the waiting list for a lung?

A

30-40% die on waiting list

29
Q

What is survival rate for lung transplantation?

A

70-80% at 5 years

50% at 10 years

Gradual attrition due to bronchiolitis obliterans