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Respiratory > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What type of condition is cystic fibrosis?

A

Autosomal recessive

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2
Q

What does cystic fibrosis result in?

A

Increased viscosity of secretions (e.g. lungs and pancreas).

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3
Q

Which organs are classically affected in someone with CF?

A

Pancreas and lungs

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4
Q

What is cystic fibrosis due to?

A

A defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel

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5
Q

What does the CFTR gene code for?

A

A cAMP-regulated chloride channel

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6
Q

What are 80% of the cases of CF in the UK due to?

A

Delta F508 on the long arm of chromosome 7

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7
Q

How many people are carriers of the CF gene?

A

1 in 25

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8
Q

How many people have cystic fibrosis?

A

1 in 2500

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9
Q

How does CF typically present in neonates?

A

Meconium ileus
Failure to thrive
Rectal prolapse

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10
Q

What is meconium ileus?

A

Not passing meconium within 48 hours, abdominal distention and vomiting.

(meconium blocks the last part of the baby’s small intestine (ileum). Above the blockage, the small intestine becomes enlarged as it fills with everything the baby swallows.)

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11
Q

How can CF present in older children/ adults?

A
  • Recurrent chest infections
  • Malabsorption- steatorrhoea, failure to thrive
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12
Q

How is CF screened in new borns?

A

Bloodspot test

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13
Q

When is the newborn bloodspot test done?

A

Day 5-9

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14
Q

What is the gold standard for diagnosing CF?

A

Sweat test

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15
Q

How can CF present in later childhood and early adulthood?

A
  • short stature
  • diabetes mellitus
  • delayed puberty
  • rectal prolapse
  • nasal polyps
  • male infertility, female subfertility
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16
Q

Why can patients with CF get rectal prolapse?

A

Due to bulky stool

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17
Q

What is abnormal about a patient with CF’s sweat?

A

Abnormally high sweat chloride

18
Q

What level of chloride is normal in a patients sweat?

A
  • normal value < 40 mEq/l,
19
Q

What level of chloride would you expect in a patient with CF?

A

> 60 mEq/l

20
Q

Which organisms may colonise a patient with CF?

A
  • Staphylococcus aureus
  • Pseudomonas aeruginosa
  • Burkholderia cepacia*
  • Aspergillus
21
Q

What is the main management of CF?

A

Chest physiotherapy and postural drainage (2x day)

22
Q

What diet is recommended for patients with CF?

A

High calorie diet, including high fat intake

23
Q

What should patients with CF try to minimise?

A

Contact with other CF patients to prevent cross infection

24
Q

What are patients with Cf at risk of cross infection of?

A

Burkholderia cepacia complex and Pseudomonas aeruginosa

25
Q

What do CF patients take long term prophylaxis for?

A

Staph Aureus infection (take flucloxicillin)

26
Q

What enzyme supplementation might patients with CF need to take?

A

Pancreatic enzyme supplementation (CREON)

27
Q

What enzyme supplementation might patients with CF need to take?

A

Pancreatic enzyme supplementation

28
Q

How can Pseudomonas aeruginosa be treated?

A

Nebulised antibiotics such as tobramycin

29
Q

How often do patients with CF need to be followed up?

A

Every 6 months

30
Q

What does the mutation on chromosome 7 result in?

A

Increased sodium absorption and abnormal chloride secretion in the epithelial cells lining the airways.

31
Q

What are the 3 main neonatal features of cystic fibrosis?

A

Failure to thrive
Meconium Ileus
Rectal prolapse

32
Q

What are the respiratory features of CF?

A

Nasal polyps
Recurrent chest infections
chronic sinusitis

33
Q

What does pancreatic insufficiency commonly result in?

A

Diabetes mellitus
Steatorrhea

34
Q

What bedside tests would you carry out in someone with CF?

A

Glucose tolerance test
Spirometry

35
Q

What type of pattern would spirometry show in someone with CF?

A

Obstructive

36
Q

What liver disease might occur in someone with CF?

A

Cirrhosis
Portal hypertension

37
Q

What is a possible MSK complication of CF?

A

Osteoporosis

38
Q

What is the management of pancreatic insufficiency?

A

Insulin replacement regime
Exocrine enzymatic replacement (Creon)
Vitamin A, D, E, K

39
Q

What is the management of liver dysfunction in someone with CF?

A

Ursodeoxycholic acid

40
Q

What is the management of worsening progressive lung disease in CF?

A

Oxygen
Non invasive ventilation
Diuretics if signs of cor pulmonale

41
Q

What might be you able to see on a chest x-ray of someone with CF?

A

Hyperinflation
Bronchiectasis

42
Q

What might you be able to see on an USS of someone with CF?

A

Distal Intestinal Obstruction
Liver cirrhosis
Chronic pancreatitis

Respiratory (47 decks)

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