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Respiratory System > cystic fibrosis > Flashcards

cystic fibrosis Flashcards

1
Q

is cystif fibrois autosominal dominant or recessive

A

recessive

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2
Q

what is the chance of getting cf if both parents are carriers

A

1/4

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3
Q

what ratio of hte population are cf carriers

A

1 in 25

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4
Q

what is the most common mutation in cf

A

f508

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5
Q

what percent of indivuls with cf will die as a result of that condition

A

90%

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6
Q

why does cf occur

A

mutation in the transmembrane conductance regulator proin cftr - this is coded on protein 7

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7
Q

what are the results of an abonoal protein channel in cf

A

clorien is trapped in teh cell, this is followed by water and sodium enter the cell.
This dehydraes the air surface liuqid and mucosu layer
this also causes a fhtick musoul surface which cause shearing
this si difficult to cough
this mucous collects bacteria reducing their ability to fight infection

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8
Q

what is the most common cftr proitin mutation

A

deltaf508

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9
Q

what ar the more sever classes of cftr mutation

A

1 -3wh

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10
Q

what are the less severe classes of cftr muations

A

4-6

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11
Q

what is antenatal testing

A

before birtha

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12
Q

what are the 3 ways of antenal testing

A

amniocentesis, choronic villous sampling , pre implantatoin genetic diagnosis

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13
Q

what blood test is done on babys life to test for cf

A

guthrie test

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14
Q

what test should be followed up is a guthrie test is positive

A

test seate to meause clorine

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15
Q

what is the cut off for the sweat thest

A

greater than 60millimoles per litere - highly likles
between 30-59millimoles per litere - possible
less than 30 millimoles per litter unlklye to be cf

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16
Q

what people are sweat test oftne not reliable in

A

audlt and infatns less than 6 months

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17
Q

what are some of the main systme stha re effected by cf

A

gi and livr - pancrati insullicanty, diabites, reflux, coeliac diase, malabosbitoin, meconium ileus
nasal - sinuses
lungs - infection and bronciapls, pneumothgoarx, infection, cor pulonaae
reporudion - infertile and delayed puberty , difficulty during pregnancy

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18
Q

what is meconium ileus

A

babys stool blocking small intestine

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19
Q

what is the effect of pancrease of cf

A

can’t produe enzyes to digast food, ths leads to abnomarl stools which are pale and offensive, float and failure to thrive in the child

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20
Q

what is the effect of growth on a cf indiuval

A

reduced growth due to cf and falling behind on hight centiles

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21
Q

what effects are the 1 - 3 classes on the pacnrease of cf

A

pancreatif insufficent

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22
Q

what effects are teh 4-6 classes on the pancreas

A

some pancreatic fucntion

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23
Q

what are the main tow emyes produefed by pancrease

A

trypsin and colistin

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24
Q

what is thed differn in pancreatif functionbetween classes 4, 5 and 6

A

incrases function

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25
Q

what are the common chest probems seen with cf

A

pneumonia, bronchiectasis, scarrring and abscesses

26
Q

what factors make pulmonary infection more common with cf

A

abnormal electorlite transport across cell memar
dehyratono fo airawysure increase amount of moucs
reduced mucos clerase
shearing of cell surface by mucous
reduced bacteria killing ability

27
Q

effect of cf on ciliated cells

A

signficaialy redued due to fewer cells present

28
Q

what is the reguccent cycle in cf

A

reduced mucoicllary clrea, bacteria adhees , reduced endocytos of bacter
colonisatoin
inlamtion mucous plugging,aiway urlection and airway damage
bronchiascis

29
Q

what is visble in cf chest xray

A

tramlinies, signet rings, mucous plugging, consolidation

30
Q

what are the clincila signs of cf

A

Low weight or height on growth charts
Nasal polyps
Finger clubbing
Crackles and wheezes on auscultation
Abdominal distention

31
Q

what is hte treatmet of pancreatic insuficently in cf

A

replae eymes uging creon, high callier diet, take nutritial supplements

32
Q

what is hte best way to treat mucous obsutcion and inflation in airway

A

airway clrease theray, mucolytics, bronchodialtors

33
Q

what is the best way to treat infection in cf

A

antibiotics,

34
Q

what is the bet way to reduce inflammation in cf

A

azihromycin - in low dose

35
Q

what is the treatemetn for scarring/ fibrosis/ bronchias in cf

A

supportive treatment and management of symptons

36
Q

what conditions are cf patine more liely to divelp

A

oseoprosis, diabetes, pneumothorax, haemoptysis

37
Q

what type of diabiets is seen in cf

A

type 2 where no insultin is made

38
Q

why are cf indivulas more at risk of diabeties

A

complies with diate, need high caleriy diet,

39
Q

what causes osteoprois in indivulas with cf

A

nutriens, steriods, delayed pubery, bad overal health.

40
Q

what are predicotrs of low bone density in cf

A

low fev1, frequit antibiotics, sterisio, low bmi, lowe exercis, ag,e males, diabeits, vigt d defcity, delayed puberty

41
Q

what percent of cf paties have a pneumo thorax

A

3-4%

42
Q

what percent of patiens have hamopths

A

about 60%

43
Q

what are the risk foacors for hameothysi in cf

A

severity, high no. of exacertbatuoin, funcal lung infection, liver disease, it k defficty, anticoagulantxs

44
Q

what are some soical and eduction and econokci impacts of cf

A

diffiult ot make frieins
missing school
talking large amounts of medications
siblings
increased time of work

45
Q

what are the no of drugs taht often are taken by cf indiucalt

A

oftne up to 30-40 dffernt types of drugs, with creon alson up t 30 to 40 capsules a day

46
Q

what are teh most common bacterial pathongs in childhood with cf in moreder

A

Stahpycolucal aureaus ( about 40%), pseudomonas aeruginosa (about 30%, haemophilius influeza, stenotrophomonas malophila, burkholderia crepia2

47
Q

what are the most common bacterial pathognes in adults with cf in order

A

psuedoman aerugaois ( about 80%) stpahcous aues ( about 30%) , haemophillusinfliez, burkhoder capera, stenotrophomonas maltophilia

48
Q

what is psuedomonas aeroginosa effects in cf

A

a bacteria which when coliased is very hard to eraiscae form cf patients as they are often become antibiotic resitan

49
Q

what percent of cf have pa in
under 1 year
over 18 year

A

under 1 - 21%
over 18 - 80%

50
Q

what is burkholderia cepacia

A

a bacateria which signicaly redues life expas and decrease lung function, it is resitant to most ant bitoics

51
Q

where does burkholderia cepacia come from

A

onioin rot and other cf patients

52
Q

what is burkholderia cenocepacia genomovar III

A

a very leaf for of burholdiea for people with cf found in bathrowmma dn soil

53
Q

where is psuedomas aeroginosa foud

A

enviroment, and other cf paties

54
Q

what cf bactria is controdictory to lung transplantation

A

non tb, mycobacteria and genomovar 111

55
Q

what are some new drugs on the market for cf

A

kaftrio

56
Q

what is the criter neded for double lung traplant

A

less than 30%fev1, estimated life survial les than 2 years , rapky detteraly lung function, pneuotha or severe haemophysis

57
Q

what can be an contral indicator to double lung transplant

A

active sytemic finction, drug/ alchol addictioms , malignaicy within 5 years, other orag fialure

58
Q

what ar some contral indicators which may still allow doule lung trospa with clinical judgement

A

orther organ dysfuction, non compliance, sterioids - greater than 20mg dialiy, absencoc of recoginsed social support, osteopros, low or high bmi, surgical risks

59
Q

what is the survail for a double lung plant after 5 and 10 years with cf

A

62% and 52%

60
Q

what are addition supprot people can be on with cf,

A

oxygen, exercize and anav care plans

61
Q

what is the comon inhertied deas in n eupropes

A

cf

62
Q
A

Respiratory System (28 decks)

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