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Respiratory System > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Cause of cystic fibrosis

A

Genetic condition - mutation of the CFTR gene on chromosome 7

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2
Q

The CF condition is

A

Autosomal recessive

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3
Q

Presentation of CF

A 
Recurrent chest infections
Wheezing
Crackles 
Shortness of breath
Bronchiectasis 
Haemoptysis
Jaundice
Failure to thrive
Foul porridge like offensive loss stools
Diabetes
Infertility in males
Liver failure
Pancreatic insufficiency
Nail clubbing
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4
Q

Investigations of CF

A

Gurthrie test/hell prick test (genetic test)
Sweat (chloride) test
Newborn screening
Antenatal testing

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5
Q

Chloride concentration <40mmol/L

A

Normal

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6
Q

Chloride concentration 40-60mmol/L

A

Borderline or intermediate

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7
Q

Chloride concentration >60mmol/L

A

Abnormal

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8
Q

Management of CF

A 
Nutritional supplements with enteric coated pancreatic enzyme supplements 
Chest physiotherapy
Sputum viscosity reduction
Genetic counselling 
Ivacaftor (drug)
Transplantation
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9
Q

Respiratory failure cause of death in what percentage

A

90%

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10
Q

CFTR gene codes for

A

Transmembrane conductance regulator

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11
Q

Mutation in CFTR results in

A

Abnormal transport of chloride and sodium

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12
Q

Result of abnormal transport of chloride and sodium

A

Reduced airway surface liquid
Thick sticky mucous
Shearing
Impaired bacterial killing via neutrophils

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13
Q

Commonest mutation class

A

Class II - delta F508 (87%)

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14
Q

Result of mutation in delta F508 on CFTR

A

Block in processing

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15
Q

Chronic villous sampling

A

Removing and testing small sample of cells from placenta

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16
Q

Amniocentesis

A

Removal and testing of amniotic fluid

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17
Q

Antenatal screening methods

A

Pre-implantation eugenic diagnosis
Chronic villous sampling
Amniocentesis

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18
Q

Neonatal screening

A

Newborn bloodspot day 5 Guthrie test

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19
Q

Pancreatic insufficiency leads to

A

Abnormal stools - malabsorcbing
Failure to thrive
Diabetes

20
Q

Percentage of CFTR function required to have sufficient pancreatic function and be asymptomatic

A

5%

21
Q

Pancreatic function in classes 1-3

A

Insufficient

22
Q

Pancreatic function in classes 4-6

A

Sufficient

23
Q

Recurrent bronchopulmonary infections

A

Pneumonia
Bronchiectasis
Scarring
Abscesses

24
Q

Reason for pulmonary infection recurrence in CF

A

Decrease in mucociliary clearance
Increase in bacterial adherence
Decrease in endocytosis of bacteria

25
Q

Bronchiectasis

A

Where airways become thickened, dilated and stiff resulting in retaining mucous which becomes infected

26
Q

Progressive respiratory decline occurs due to

A

Progressive bronchiectasis
Recurrent LRTIs
Progressive airflow obstruction
Respiratory failure

27
Q

Presentation in CT scan

A

Tramlines
Signet rings
Mucous plugging
Consolidation

28
Q

Treatment of pancreatic insufficiency

A

Enteric coated enzyme pellets
High energy diet
Fat-soluble vitamin and mineral supplements

29
Q

Treatment of mucus obstruction and inflammation

A

Airway clearance via physiotherapy
Mucolytics
Bronchodilators

30
Q

Treatment of chronic infection

A

Antibiotics (oral, IV or nebulised)

31
Q

Treatment of increased inflammation

A

Azithromycin

32
Q

Treatment of fibrosis/scarring/bronchiectasis

A

Supportive treatment and management of symptoms

33
Q

Aspects of CF to be aware of

A

Diabetes
Osteoporosis (BMD)
Pneumothorax
Haemoptysis

34
Q

Type of diabetes in CF

A

Type 2

35
Q

Management of diabetes in CF

A

Joint diabetic/CF clinics

CF dietician

36
Q

Treatment of osteoporosis

A

Bone protection medication

Weight bearing exercise

37
Q

Reason for osteoporosis

A 
Malnutrition and low BMI
Steroids
Delayed puberty
Inflammatory cytokines in sepsis
Vit D/K deficiency
38
Q

Reason for haemoptysis

A

Bronchial wall destruction

39
Q

Risk factors of haemoptysis

A 
Severity of CF
Exacerbations
Fungal lung disease
Liver disease
Vit K deficiency
Anticoagulants
40
Q

Massive haemoptysis

A

> 240mls in 24 hours or 100mls/hour

41
Q

Management of massive haemoptysis

A

Admit
Resuscitate
Bronchial angiogram
Embolisation

42
Q

Common microbiology in childhood

A

Staph aureus

Haemophilus influenzae

43
Q

Common microbiology in adulthood

A

Pseudomonas aeruginosa

44
Q

Less common microbiology

A 
Burkholderia capacia
Aspergillus
Strenotrophomonas
MRSA
Atypical mycobacteria
45
Q

Pseudomonas aeruginosa

A

Colonisation increase with age

Reduces life expectancy and rapid decline lung function

46
Q

If not colonised by bacteria the median survival is around

A

40

Respiratory System (20 decks)

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