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medical microbiology > Cystic Fibrosis 3 > Flashcards

Cystic Fibrosis 3 Flashcards

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USMLE® Step 1 flashcards Biology 101 flashcards
1
Q

What is the most common autosomal recessive disorder in caucasians?

A

Cystic fibrosis

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2
Q

How many people have the CF gene?

A

1:25

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3
Q

How many people suffer from CF?

A

1:2500

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4
Q

Why has survival of CF gotten better?

A

CF centres

MDT teams

Physio

Nutrition/enzymes

Antibiotics

Aggressive approaches

Annual flu vaccine

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5
Q

What is the life expectancy for someone with CF?

A

37 years

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6
Q

Where is the CF gene located?

A

Chromosome 7

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7
Q

What does a mutation of the transmembrane conductance regulator protein cause?

A

Abnormal transport of chloride and sodium across epithelium

Reduced chloride secretion from epithelium

Reduced sodium absorption form lumen

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8
Q

What does a mutation of the CF gene lead to?

A

Thick secretions and impaired bacterial killing via neutrophils as normal chloride is required

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9
Q

What is the heterozygous advantage of CF?

A

Mice resistant to salmonella and cholera toxin

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10
Q

How many classes of mutations of the CF gene are there?

A

6

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11
Q

What percentage of CFTR function do you need to have sufficient pancreatic function and be asymptomatic?

A

5%

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12
Q

What are some challenges for patients with CF?

A

Transition

Prognosis

Promise of new drugs

Possibility of lung transplant

Other conditions

Media/social media pressures

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13
Q

What are some other conditions that can co-incide with CF?

A

Diabetes

Liver disease

Osteoporosis

Fertility issues

Haemoptysis

Mental health issues

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14
Q

What are the presentations of CF in adults?

A

Infertility in men

Bronchiectasis

Recurrent infections

Hyperinflation

Clubbing

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15
Q

What does CF lead to pulmonary infection?

A

Decrease in mucociliary clearance

Increase in bacterial adherence

Decrease in endocytosis of bacteria

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16
Q

What are some respiratory problems that CF can cause?

A

Progressive bronchiectasis

Recurrent lower respiratory tract infections

Progressive airflow obstruction

Respiratory failure

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17
Q

How is diabetes associated with CF?

A

Type 1 rarely seen

Type 2 often preceded for years by falling lung function

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18
Q

What issues coming with being diabetic and having CF?

A

Compliance with diet

Low sugar/high fibre diet not appropriate

OGTT/HbA1c used but not perfect

Insulin of benefit, but not so much oral hypoglycaemics

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19
Q

What does OGTT stand up for?

A

Glucose tolerance test

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20
Q

What does HbA1c stand up for?

A

Average blood glucose levels for the last 2 to 3 months

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21
Q

What is osteoporosis?

A

Condition that weakens the bones, making them fragile and more likely to break

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22
Q

What is a condition that weakens the bones, making them more fragile and likely to break called?

A

Osteoporosis

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23
Q

How does osteoporosis affect bone remodelling?

A

Slower gain, faster loss

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24
Q

What may osteoporosis exclude?

A

Lung transplant

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25
Q

What could osteoporosis occur due to?

A

Malnutrition and low BMI

Steroids

Delayed puberty and hypogonadism

Inflammatory cytokines from sepsis

Vitamin D/K deficiency

Drugs

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26
Q

What vitamin deficiency can lead to osteoporosis?

A

Vitamin D and K

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27
Q

What does BMD stand up for?

A

Bone mineral density

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28
Q

What are predictors of a low bone mineral density (BMD)?

A

Low FEV1

Steroids

Low BMI

Low exercise

Age

Male

Diabetes

Vitamin D

Delayed puberty

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29
Q

What percentage of CF patients get a pneumothorax in their life?

A

3-4%

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30
Q

How many CF patients are older than 18 years old?

A

50%

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31
Q

What is the chance of a pneumothorax reoccurance for CF patients?

A

50%

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32
Q

What is the treatment for CF patients with a pneumothorax?

A

Same as normal (drain, pleurodesis, surgery)

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33
Q

What is haemoptysis?

A

Coughing up blood

34
Q

What is coughing up blood called?

A

Haemoptysis

35
Q

Why does haemoptysis occur?

A

Bronchial wall destruction

36
Q

What are the risk factors of haemoptysis?

A

Severity

Exacerbations

Fungal

Liver disease

Vitamin K deficiency

37
Q

How do the typical types of bacteria that cause infections change with age?

A

Different ones cause a greater proportion at different ages

38
Q

How does colonisation of pseudonomas aerugosa change with age?

A

Colonisation increases with age

39
Q

Where is pseudonomas aeruginosa acquired from?

A

Environment (particularly hospitals)

Other CF patients

Segregation and disinfection policies

40
Q

What is colonisation of pseudonomas aeruginosa associated with?

A

Reduced life expectancy (28 years)

Rapid decline in lung function

41
Q

What can be said about pseudonomas aeruginosa and antibiotic resistance?

A

Rapidly acquired antibiotic resistance

42
Q

What is the life expectancy of someone with CF who has a colonisation of pseudonomas aeruginosa?

A

28 years

43
Q

What does pseudonomas aeruginosa produce?

A

Toxins that destroy bronchial epithelium

44
Q

Where is burkholderia cepacia acquired from?

A

Environment

Other CF patients

Segregation policies

45
Q

What is colonisation of burkholderia cepacia associated with?

A

Reduced life expectancy (16 years)

Rapid decline in lung function

Do worse in pregnancy

46
Q

What is the life expectancy of someone with CF and a colonisation of burkholderia cepacia?

A

16 years

47
Q

What can be said about burkholderia cepacia and antibiotic resistance?

A

Innate resistance to most antibiotics

48
Q

When does colonisation of sternotrophonas maltophilia occur?

A

Usually after pseudomonas but can occur as first gram negative infection

49
Q

What can be said about sternotrophonas maltophila and antibiotic resistance?

A

Multiple antibiotic resistance

50
Q

How does sternothrophonas maltophilia affect the CF prognosis?

A

Unsure if detrimental to prognosis

51
Q

Where is aspergillus acquired from?

A

The environment not person to person

52
Q

What percentage of CF patients is atypical mycobacterium?

A

13%

53
Q

What is the most common atypical mycobacterium found in CF patients?

A

Mycobacterium abscessus (75% of mycobacterium)

54
Q

When should atypical mycobacterium be treated?

A

If clinical/lung function/chest X-ray deteriation

55
Q

What can be said about transplanation for patients with mycobacterium abscessus?

A

Conta-indication

56
Q

What does eradication of mycobacterium abscessus require?

A

1 month in the hospital then maintenance treatment for 1 year which has side effects and can lead to failure

57
Q

What is involved in the management of a pulmonary infection?

A

Treat early and aggresively with antibiotics

Oral antibiotics (staph, haemophillus, pneumococcus)

IV antibiotics (strenotrophomonas, burkholderia)

58
Q

For what organisms should oral antibiotics be used for pulmonary infections?

A

Staph, haemophilus, pneumococcus

59
Q

For what organisms should IV antibiotics be used for pulmonary infections?

A

Stenotrophomonas, burkholderia

60
Q

What should be done if the organisms causing a pulmonary infection are multiple resistant?

A

Test for synergy between antibiotics

61
Q

Why do some CF patients use bronchodilators?

A

They have an airway obstruction

62
Q

Why might CF patients have an airway obstruction?

A

Asthma/atopy

Mechanical (bronchhial plugging, inflammation)

63
Q

What is airway clearance done by?

A

Chest physio

64
Q

What does chest physio involve?

A

Autogenic drainage

Active cycle of breathing

Huffing

Airway oscillating devices (PEP device)

Percusion vests

65
Q

What are mucolytics?

A

Drugs that reduce viscocity of phelgm

66
Q

What are drugs that reduced viscosity of phelgm called?

A

Mucolytics

67
Q

What are examples of mucolytics?

A

Pulmozyme

Hypertonic saline

Carbocysteine

Bronchitol

68
Q

What effects does azithromycin have?

A

Anti-inflammatory and antibacterial

69
Q

What does treatment with azithromycin do?

A

Increases lung function

Decreases lung decline

Decreases exacerbation rate

70
Q

What are new drugs for CF called?

A

Potentiates and correctors

71
Q

What do potentiates and correctors do?

A

Address different processes in CFTR production, processing, folding, transport and insertion into the membrane

72
Q

What can you say about the price of potentiates and correctors?

A

They are very expensive, with some costing more than £100,000 per year per patient

73
Q

What are examples of potentiates and correctors?

A

Ivacaftor

Ivacaftor/lumbacaftor (orkambi)

Tezacaftor/ivacaftor (syndeco)

74
Q

What is orkambi a combination of?

A

Ivacaftor/lumbacaftor

75
Q

What is syndeco a combination of?

A

Tazacaftor/ivacaftor

76
Q

What are indications for lung transplantation?

A

Rapidly deteriorating lung function

FEV1 < 30% predicted

Life threatening exacerbations

Estimated survival < 2 years

77
Q

How many people die on the waiting list for a lung transplant?

A

30-40%

78
Q

How many people survive 5 years after a double lung transplant?

A

70-80%

79
Q

How many people survive more than 10 years after a double lung transplant?

A

50%

80
Q

What are things that can improve quality of life?

A

Lung transplant

Oxygen

Exercise

Support

Advanced care planning

medical microbiology (29 decks)

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