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Mucosal Surfaces > 27- CF 1 > Flashcards

27- CF 1 Flashcards

1
Q

What sort of defect is cf

A

Recessive autosomal defect in CFTR gene expressed on many organs

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2
Q

What are the stats in uk

A

10k people have it and 1 in 2500 babies

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3
Q

What does multisystem disease mean

A

Affects the lungs, liver, bones, gut, heart and pancreas / other areas due to CFTR expression

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4
Q

What sort of things does it cause in the lungs

A

Bronchitis, bronchiectasis, pneumonia and resp faulrure

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5
Q

What sits on top of Eli cells in normal airway

A

Airway surface liquid made from 2 layers and top layer is more mucusy vs ciliary layer on lower side

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6
Q

What does CFTR normally do affecting asl

A

Allows hydration through secretion of Cl and HCO drawing water out into airway for better mucus clearance

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7
Q

What are the 3 hallmarks of Cf

A

Infection,mucus and inflammation via inflam cells

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8
Q

Which article discusses effects of CFTR on airway surface and neutrophils

A

Mall 2014

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9
Q

Which channel does CFTR regulate so not too much na hyper absorption occurs and allows hydration

A

Enac

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10
Q

Does mucus building through dehydration always need to cause infection to cause inflammation

A

No. Some irritants can cause pro inflam cytokines and chemokine release without infectikn

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11
Q

What sorts of things can NE cause

A

Bronchiectasis, mucus hypersexretion, airway inflammation and cell damage

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12
Q

How does ne also cause more dehydration so more mucus build up

A

Activates the enac channel and because CFTR not functioning can’t regulate this = dehydration eg phe508 mutation

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13
Q

Reduced ph occurs with less HCO release. What does this impact on

A

Amp, lysozymes and lactoferrins action to remove pathogens

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14
Q

Which 3 things can help secretion clearance to manage cf

A

PEP (positive exp pressure) Physiotherapy

Exercise

Mucolytics / other mucoactives

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15
Q

How does pep physiotherapy work

A

Keepsairways open and better ventilation through a machine so more clearance

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16
Q

How would you treat the inflammation

A

Azithromycin or steroids

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17
Q

Why was cf first seen as a disease of the gut

A

CFTR on pancreas allows fat absorption so this dysfunction reduces fat soluble vitamin absorption eg vit a, e,d,k

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18
Q

What sorts of signs are fat malabsorption

A

Greasy stools, weight loss

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19
Q

What obstruction of the bowel is a sign of cf in babies or adults

A

Meconium ileus (in distal ileum)

In adults it’s distal bowel obstructions

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20
Q

Which article discusses the vitamin deficiency

A

Rayner 1992

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21
Q

What does vit k deficiency cause in cf patients

A

Haemorrhagic disease (important for clotting)

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22
Q

What does d deficiency cause

A

Osteomalacia, sometimes rickets and bone deformation (important for bone development)

23
Q

What is E linked with to do with neurological problems

A

Loss of position and vibration sense in lower limbs, also muscle weakness and tremors

24
Q

How does vitamin E link to inflammation in airway in cf

A

If present it usually blocks free radical caused inflammation eg from neutrophils

25
Q

What does A deficiency cause to do with eyes

A

Night blindness

26
Q

What is seen in the airway during vitamin a deficiency

A

Increased bacterial adherence to epi cells

27
Q

Which article discusses meconium ileus

A

Sathe 2017

28
Q

What does lowered ph allow to bind the mucus

A

Albumin and protein bound carbohydrates which cause obstruction in the terminal ileum

29
Q

What % of Cf have this

A

20%

30
Q

What CFTR class mutations is it due to

A

Class 1-3

31
Q

Which therapy helps pancreas absorb fats helping vitamin absorption

A

Pert. Pancreatic enzymes replacement therapy

32
Q

How are vitamins given for supplementation

A

Oil based

33
Q

What is gastrostomy feeding

A

Feeding tube links to stomach for better vitamin supply

34
Q

What reflux disease caused by CFTR disfunction needs treated

A

Gord

35
Q

Why does dysbiosis of gut occur in cf

A

Mucus alteration and ph changes due to CFTR and inflammation due to no aea

36
Q

What sorts of bacteria are reduced

A

Bacteroides and firmicutes

37
Q

What do bacteroides help

A

Reduce il8 and therefore neutrophil inflammation eg b fragiliswhich binds tlr2 and causes il10 treg upreg (round 2011)

38
Q

Why is diabetes associated with cf

A

There is destruction of islet cells in pancreas so reduced insulin

39
Q

What is ABPA caused in cf

A

Allergic bronchopulmonary aspergillosis (allergic reaction eg with igE and eosinophils in airway to aspergillus ag)

40
Q

How does cf affect reproduction

A

Infertility in men

41
Q

What sort of things are looked for in the clinical diagnosis

A

Chest infection/ resp issues, malabsorption, weight loss, liver issues eg jaundice , meconium ileus

42
Q

What is the newborn screening method

A

Heel prick to take blood sample screened for 9 diseases inc cf

43
Q

What levels suggest possible cf so then genetically tested

A

Immunoreactive trypsinogen (irt)

44
Q

What talks about Nbs more

A

Deboeck et al 2017

45
Q

What is irt sign of

A

Pancreatic injury

46
Q

Why is genetic tests needed even if high irt

A

Can be caused by perinatal stress too

47
Q

Why can sometimes cf have low irt

A

Meconium ileus associated

48
Q

What is the sweat test done on children

A

Measure levels of Cl release which is abnormally high in cf from sweat glands

49
Q

Why does CFTR mutation cause increase na and Cl in sweat

A

Hypertonic sweat as CFTR usually absorbs more na and Cl in the sweat glands

50
Q

Levels above whay are cf indication

A

60mmol/L

51
Q

A few other tests are done to diagnose newborns if other ways don’t work eg sweat test. How

A

Bronchoscoph/Bal washing and also sputum analysis after cough swabs done

52
Q

What does the issue of heterogeneity mean in Cf

A

Some cases missed as many signs eg abpa, asthma and bronchiectasis and pancreatitis overlooked for cf

53
Q

What is done for inconclusive results ie below 60mmol/L

A

Put into groups of risk eg high risk would be managed more often/visit more often

54
Q

What is the low risk group vs medium risk

A

30Cl or below levels so see them annually

Medium risk is 30-60 so see them every 6 months

Mucosal Surfaces (33 decks)

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