3 CF, lung cancer and OSA Flashcards

1
Q

How do you get CF

A
  • inherited disorder
  • CFTR gene (chromosome 7)
  • recessive
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2
Q

What is CF

A

Inherited defect in cell chloride channels

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3
Q

What organs are particularly affected in CF

A
  • Lung congestion

- Malabsorption of nutrients by the pancreas

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4
Q

How do they diagnose CF

A
  • Perinatal testing (all children now screened -blood)

- ‘sweat test’ - measures salt content of sweat as greater salt in CF patients

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5
Q

symptoms of CF

A
  • troublesome cough
  • repeated chest infections with unusual organisms e.g. psuedomonas
  • prolonged diarrhoea
  • poor weight gain (can’t digest fat)
  • liver dysfunction
  • prone to osteoporosis
  • diabetes symptoms
  • reduced fertility
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6
Q

treatment of CF

A
  • physiotherapy
  • medication
  • exercise
  • transplantation
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7
Q

How is physiotherapy used in CF treatment

A

to remove mucous secretions from the lungs, performed at home by parents

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8
Q

How is medication used in CF treatment

A

Lungs:

  • bronchodilators to open airways
  • antibiotics to reduce chest infection frequency
  • steroids to reduce airway inflammation
  • Dnase to break down mucus

Digestive system:

  • pancreatic enzyme replacement
  • nutritional supplements
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9
Q

How is exercise used in CF treatment

A
  • to keep lung function optimal

- to build physical bulk and strength

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10
Q

How is transplantation used in CF treatment

A
  • not a cure
  • end stage lung disease (also affects heart)
  • heart-lung transplant (better)
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11
Q

How can gene therapy be used as CF treatment

A

its easy to get gene into adult cells because use a virus as a carrier to put into cells
Problem is when cells are dying or replaced they are replaced by the same faulty cell in the first place, getting progressively better

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12
Q

What proportion of lung tumours are malignant

A

almost all + spread

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13
Q

what is the most common lung cancer

A

squamous cell carcinoma

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14
Q

what causes squamous cell carcinoma

A

smoking

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15
Q

what are the different types of lung tumour

A
  • squamous cell carcinoma
  • small cell
  • large cell
  • adenocarcinoma
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16
Q

What lung tumour is most linked to smoking

A

squamous cell carcinoma

17
Q

What is a big problem of lung tumours

A

the don’t unusally present until quite far down the line

18
Q

What effects do lung tumours have

A
  • cough
  • haemoptysis (normal sputum with strips of blood)
  • pneumonia
  • metastasis
  • dysphagia (tumour pushing against oesophagus)
  • svc obstruction
  • recurrent laryngeal nerve palsy (one side gets paralysed -embryology)
19
Q

What is the difference of the sputum between heart failure and lung tumours

A

heart failure = pink frothy sputum

lung tumour = normal with strips of blood

20
Q

how long does it take for a lung tumour to develop

A

a long time

21
Q

what is key to preventing lung tumours

A

smoking cessation

22
Q

where do squamous cell carcinomas and non-squamous cell carcinomas tend to be in the lungs

A

squamous cell carcinoma - centrally

others - peripherally

23
Q

What is the survival rate like for lung tumours

A

75% die within 1st year after diagnosis

24
Q

What does OSA stand for

A

obstructive sleep apnoea

25
Q

what is OSA

A

airway obstruction whilst asleep for 10secs or more

26
Q

what happens during OSA

A

airway muscle tone drops

  • soft palate and tongue flops back
  • both completely occlude airway
  • normally you move your tongue but with OSA you can’t
  • they wake up regularly as build up of CO2
27
Q

what are characteristics or sleep apnoea

A
  • sometimes snorers sometimes not
  • drowsiness during the day
  • increased risk of MI (low oxygen so chronic hypoxia at night)
28
Q

what is more common, OSA or central sleep apnoea

A
  • OSA is most common

- mixed form possible

29
Q

How do you measure OSA

A

apnoea/hypopnea index (score <10 few problems)

30
Q

Treatment of sleep apnoea

A
  1. Continuous positive airway pressure (CPAP)
    - blows air into upper airway, enough just to keep tongue and airway apart
    - noisy
    - will make them snore more
  2. mandibular advancement devices
    - tongue attached to mandible
    - pull mandible forward with gum shield
    - uncomfortable