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cycstic fibrosis Flashcards

1
Q

what is the prevalence of cystic fibrosis

A

1 : 2,500

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2
Q

how does CF effect patients fertility

A

men - 95% infertile

females are fertile - though poor nutritional sate may hinder that

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3
Q

what are the options for the infertile male CF patients

A

intra-cytoplasmic sperm injection (30% success rate)

adoption

sperm donor

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4
Q

what are the contraindications for CF lung transplant

A

other organ failure

malignancy within 5 years

significant peripheral vascular disease

addiction

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5
Q

what is the problem with lung transplant

A

there is a goldilocks zone

too good

or

too bad

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6
Q

when are CF patients bad enough

A

FEV1 <30% predicted

sever weight loss

hypoxia

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7
Q

how has CF median survival got better

A

CF centres

multi-disciplinary teams

physio

nutrition/ enzymes

antibiotics

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8
Q

what is the main cause of death in CF patients

A

respiratory failure

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9
Q

what is the pathology of CF

A

a mutation with the CFTR gene (cystic fibrosis transmembrane conductance regulator)

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10
Q

what does a mutation with the CFTR

A

problems with cilia

causing decreased mucociliary clearance and endocytosis of bacteria

increase of bacterial adherence

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11
Q

what are the presenting features of CF for neonatal

A

screening

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12
Q

what are the presenting features of CF in infants and young people

A

Recurrent chest infection

failure to thrive

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13
Q

what are the presenting features of CF in older children and adults

A

recurrent chest infections

male infertility

bronchiectasis

hyperinflation

clubbing

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14
Q

what is the neonatal screening for CF

A

new-born bloodspot day 5

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15
Q

how do you make a CF diagnosis

A

clinical assessment
sweat test

repeat CF mutation analysis

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16
Q

what is CF in relation to the rest of the body

A

it is a multi system disease

17
Q

what is one system that is often damaged by CF

A

pancreatic insufficiency

85 - 90%

abnormal stools

18
Q

how is pancreatic insufficiency from CF treated

A

enteric coated enzyme pellets

high energy diet

fat soluble vitamin and mineral supplements

19
Q

what are the cardinal features of CF

A

recurrent bronchopulmonary infections

bronchiectasis

fibrosis

scarring

20
Q

what is the vicious cycle of CF

A

mucus obstruction

leads to chronic infection

leading to increased inflammation

leading to fibrosis, scarring, bronchiectasis

21
Q

what treatments of mucus obstruction

A

airway clearance physiotherapy, hypertonic saline

22
Q

what is used to treat the chronic infections

A

antibiotics

23
Q

what is used to treat the increase inflammation

A

azithromycin

ibuprofen

24
Q

what are the common infective organism in CF

A

staph aureus

H. influenze

25
Q

what are the cornerstones for treatments

A

proactive treatment
good nutrition
active lifestyle

26
Q

where is the potential for a cure

A

CFTR modulator treatment

gene therapy/ editing

27
Q

what do images of CT show with patients that have CF

A

tramlines

signet rings (circles, with calcifications)

consolidation

28
Q

how does type two diabetes tie in with CF

A

poor lung function

leading to inactive

leading to High BMI

29
Q

what is the treatment of pulmonary infections in CF

A

treat early and aggressively

30
Q

what other treatments for generally quality of life in CF patients

A

bronchodilators

airway clearance

mucolytics

31
Q

what are some other disease that CF patients often get

A

type 2 diabetes

osteoporosis

pneumothorax

haemostasis

32
Q

what is one of the most common if not the most common infection in CF patients

A

pseudomonas aeruginosa

33
Q

what is the pseudomonas aeruginosa - how is it treated

A

acquired form environment or CF patients

reduced life expectance

rapid lung decline

attempt eradication

respiration (17 decks)

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