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Flashcards in Cystic Fibrosis 3 Deck (80)
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1

What is the most common autosomal recessive disorder in caucasians?

Cystic fibrosis

2

How many people have the CF gene?

1:25

3

How many people suffer from CF?

1:2500

4

Why has survival of CF gotten better?

CF centres

MDT teams

Physio

Nutrition/enzymes

Antibiotics

Aggressive approaches

Annual flu vaccine

5

What is the life expectancy for someone with CF?

37 years

6

Where is the CF gene located?

Chromosome 7

7

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

Abnormal transport of chloride and sodium across epithelium

Reduced chloride secretion from epithelium

Reduced sodium absorption form lumen

8

What does a mutation of the CF gene lead to?

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

9

What is the heterozygous advantage of CF?

Mice resistant to salmonella and cholera toxin

10

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

6

11

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

5%

12

What are some challenges for patients with CF?

Transition

Prognosis

Promise of new drugs

Possibility of lung transplant

Other conditions

Media/social media pressures

13

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

Diabetes

Liver disease

Osteoporosis

Fertility issues

Haemoptysis

Mental health issues

14

What are the presentations of CF in adults?

Infertility in men

Bronchiectasis

Recurrent infections

Hyperinflation

Clubbing

15

What does CF lead to pulmonary infection?

Decrease in mucociliary clearance

Increase in bacterial adherence

Decrease in endocytosis of bacteria

16

What are some respiratory problems that CF can cause?

Progressive bronchiectasis

Recurrent lower respiratory tract infections

Progressive airflow obstruction

Respiratory failure

17

How is diabetes associated with CF?

Type 1 rarely seen

Type 2 often preceded for years by falling lung function

18

What issues coming with being diabetic and having CF?

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

19

What does OGTT stand up for?

Glucose tolerance test

20

What does HbA1c stand up for?

Average blood glucose levels for the last 2 to 3 months

21

What is osteoporosis?

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

22

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

Osteoporosis

23

How does osteoporosis affect bone remodelling?

Slower gain, faster loss

24

What may osteoporosis exclude?

Lung transplant

25

What could osteoporosis occur due to?

Malnutrition and low BMI

Steroids

Delayed puberty and hypogonadism

Inflammatory cytokines from sepsis

Vitamin D/K deficiency

Drugs

26

What vitamin deficiency can lead to osteoporosis?

Vitamin D and K

27

What does BMD stand up for?

Bone mineral density

28

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

Low FEV1

Steroids

Low BMI

Low exercise

Age

Male

Diabetes

Vitamin D

Delayed puberty

29

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

3-4%

30

How many CF patients are older than 18 years old?

50%