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Respiratory system SA > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What is cystic fibrosis gene prevalence

A

1:25

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2
Q

Where does the cystic fibrosis genes lie

A

On chromosome 7

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3
Q

What is the aetiology of cystic fibrosis

A

Mutation in the gene CFTR

changing a protein that regulates the movement of salt in and out of cells

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4
Q

What is the result of cystic fibrosis

A

The result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as increased salt in sweat

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5
Q

What are examples of 5 different classification of CFTR mutations

A

1: No synthesis of CFTR channel
2: No maturation of CFTR - proteased as unrecognised
3: Blocked CFTR - inactive
4: decreased conductance of CFTR
5: decreased abundance of CFTR channels

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6
Q

What Is the basic effects CF mutation can have on the CFTR gate

A

Quantity of CFTR channels

The function of the CFTR channel - gating/conductance

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7
Q

What is the function of CFTR gate

A

active transport for chloride ions

Controlling cilla that regulates the liquid volume on the epithelia surface

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8
Q

What is the pathology of a mutated CFTR gate that leads to bronchiectasis

A

Prevents the transport of chloride ions, so no longer regulates the liquid volume on the epithelia surface

Abnormality leads to:

cilla collapse
-decreased muco-cillary clearance

  • production of a thick, sticky mucus in the respiratory and digestive system
  • increased bacterial adherence
  • Build up mucus and bacterial adherence/colonisation leads to inflammation
  • airway damage/ulceration
  • Progressive airflow obstruction
    = bronchiectasis
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9
Q

Whats is the symptoms of CF in children

A

Recurrent chest infection

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10
Q

What is the screening process for neonatal babies in the detection of CF

A

Guthrie test (heel-pin test) for day 5 babies;

  1. Initial - immunoreactive trypsinogen
  2. If positive - mutation analysis performed
  3. Screen positive referred sweat test
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11
Q

What is the management of CF

A

specialist multidisciplinary team

  • Primary care involving surveillance
  • early treatment of infection
  • Good nutrition
  • Active life
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12
Q

What is the benefits of screening for CF

A

as awareness of leads to:
reduced mortality

increased lung function

increased nutrition

Prevent lower cognitive skills - as find vitamin E deficiency in the brain

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13
Q

What is the two cardinal features of CF

A

pancreatic insufficiency - due to mucus blockage

Recurrent bronchopulmonary infection

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14
Q

What is the outcome of pancreatic insufficiency

A

failure to thrive due no absorption of fats and vitamins

Creating abnormal stools - oilly, pale, offensive

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15
Q

What is the management of pancreatic insufficiency

A

Enteric coates enzyme pellets (deal with fat)
H2 antagonists
Proton pump inhibitors
Good nutrition
- high energy diet
Fat soluble vitamin + mineral supplements
Active life

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16
Q

Why would you not give CF patient with pancreatic insufficiency a low fat diet

A

As fat is still needed in you body, and the enzyme is tailored to deal with the fat

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17
Q

What is examples of some of the Recurrent bronchopulmonary infection experienced by CF patient

A

Pneumonitis
Bronchiectasis
Scarring
Abscesses

18
Q

What Pre infection management is needed for a patient with CF to avoid recurrent bronchopulmonary infection

A 
Segregation of CF patients to prevent cross-infection
Airway clearance and adjuncts
Mucolytics - break down on mucus 
Prophylactic antibiotics 
Annual influenza vaccination
19
Q

What are common reparatory pathogen in CF

A

Early years:
Staphylococcus aureus
Haemophilus influenzae

Later years:
Pseudomonas aeruginosa 
Burkholderia cepacia 
Stenotrophomonas maltophilia 
Mycobacterium abscessus
20
Q

What is the treatment options for chronic respiratory tract infection in a CF patient

A

ANTIBIOITCS

  • Suppress bacterial load
  • Treat infective exacerbations

Reduce inflammation

21
Q

What drugs would be used to reduce inflammation in treating chronic respiratory tract infection in a CF patient

A

Ibuprofen
Azithromycin
Prednisolone

22
Q

Where else can CF manifest to

A

GI Dysmotility - muscles of the digestive system become impaired

hepatopathy - liver congestion/liver disease

Upper airway polyps and sinusitis

Diabetes

Bones - osteoporosis

Heat exhaustion

Bilateral absence of vas deferens - reproductive organs fail to work

Vaginal candidiasis; stress incontinence

fertility issues
Male infertility

23
Q

What kind of disease is CF

A

Multi -organ disease

24
Q

Where is the bacteria acquired from in CF

A

The environment

other CF patients

25
Q

What is the morphology of pseudomonas aeruginosa do once colonised, what does this cause

A

Undergoes mucoid change and forms a biofilm

then microcolonies within the film protecting itself from its host so it has high antibiotic resistance

26
Q

How do you treat pseudomonas aeruginosa

A 
nebulised colomycin
with antibiotics -
 oral ciprofloxacin 
or 
 i.v. ceftazidime (if the other fails)
27
Q

The colonisation of pseudomonas aeruginosa and Burkholderia cepacia cause

A

reduced life expectancy
rapid decline in lung function due to
‘cepacia syndrome,’

28
Q

When does Stenotrophomonas maltophilia has frequent colonisation

A

Usually after pseudomonas

but can occur as first Gram negative infection

29
Q

What respiratory bacterial pathogen is resistant to all anti tuberculosis chemotherapy

A

Mycobacterium abscessus

30
Q

Mycobacterium abscessus resistant means the best treatment option with this respiratory infection would be

A

Lung transplant

31
Q

What antibiotic administration is used for Staph, Haemophilus and Pneumococcus

A

Oral

32
Q

What antibiotics is used for Pseudomonas, Stenotrophomonas and Burkholderia, why?

A

IV - all have very high antibiotic resistance

33
Q

What specific type of antibiotic is treatment needed in CF patients

A

Large doses of two antibiotics
Blactam + amino glycoside
on a two week course

34
Q

What is the name of a new class of drugs affecting primary defect in CF and what is its mechanisms

A

Ivacaftor

binds to CFTR, improves the transport of chloride ions

35
Q

What is the advantages and disadvantages of if Ivacaftor

A

ADV
Improves lung function 10% predicted
Weight gain
Reduces sweat chloride

DIS
expensive dolla bills

36
Q

Why in a lung transplant do both lungs need to be transplanted

A

As the new lung would only be infected by the old one

37
Q

What is the indications for needing a lung transplant

A

Rapidly deteriorating lung function - hypoxia

FEV1 < 30% predicted

weight loss

Hypercapnia

Recurrent worse sepsis
Life threatening exacerbations
Estimated survival <2 years
Bad quality of life

38
Q

What is the purpose of a lung transplant

A

Not a solution
Prolong survival
and Improve quality of life

39
Q

What is the major factors that cause contradiction to lung transplant

A
  • Low BMI
  • Other organ dysfunction/failure
  • On steroids
  • Malignancy within 5 years
  • Significant peripheral vascular disease
  • Drug abuse
  • Active infections or microbiological issues
    e. g. M. abscessus
  • Surgical risks
  • osteoporosis
40
Q

What is the symptoms of CF

A

Chronic purulent sputum production

Recurrent chest infection
(pneumonitis / bronchiectasis / scarring / abscesses)

Weight loss

Fever

41
Q

What is the signs of CF

A

haemopytsis (infection)

pneumothroax (older males)

male infertility

nasal polyps

Onset of diabetes - pancreas issues

Failure to thrive due pancreatic insufficiency:

Abnormal oilly and offensive stools (steatorhea)
meconium delay in babies (first poop) 
osteoporosis
vitamin D issue 
malnutrition

Respiratory system SA (37 decks)

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