developmental aspects of lung disease Flashcards

1
Q

5 stages of lung development

A

embryonic, pseudoglandular, canalicular, saccular, alveolar

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2
Q

between what age is embryonic lung development

A

3-8wks

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3
Q

between what age is pseudoglandular lung development

A

5-17wks

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4
Q

between what age is canalicular lung development

A

16-26wks

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5
Q

between what age is saccular lung development

A

24-38wks

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6
Q

between what age is alveolar lung development

A

36wks-2/3yrs

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7
Q

embryonic lung development

A

lungs appear as offshoot from the oesophagus no clear cell differentiation yet ridge develops between oesophagus and trachea, lung buds continue to develop off, lobes can be seen developing

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8
Q

pseudoglandular lung development

A

progressive spreading of the bronchi, conducting system begins to form as sections of the lung begin to divide off cilia develop around wk 13

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9
Q

canalicular lung development

A

early gas exchange structures form lung becomes increasingly vascularised which is important in its overall development type I and II pneumocytes begin to differentiate structures involved in surfactant production are produced

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10
Q

saccular lung development

A

further evolution of the gas exchange structures type I and II pneumocytes become visible thinning out of areas for gas exchange

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11
Q

alveolar lung development

A

usually occurs after the child is born sacuoles change in shape, geometry and function after birth and continue to grow over the next 3-12 yrs

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12
Q

post-natal lung growth

A

alveolar septation continues 100-150mln at birth to 200-600mln at 3-8yrs increased alveolar dimensions thereafter anything that affects the amount of alveoli you have in early life will have a knock on effect later on

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13
Q

common upper (tracheo-bronchial) congenital abnormalities

A

tracheal agenesis and stenosis tracheomalacia tracheo-oesophageal fistula (relatively common)

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14
Q

common lower (broncho-pulmonary) congenital abnormalities

A

lung agenesis/pulmonary hypoplasia bronchogenic cyst, CPAM (sporadic malformations) congenital diaphragmatic hernia

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15
Q

how are congenital abnormalities diagnosed

A

antenatally newborn childhood asymptomatic - incidental finding

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16
Q

presenting features antenatally

A

US scan 12 wk dating scan - abnormalities are more likely to be picked up at 20wks fluid amount can indicate problems w/ lungs or gut can do MRI to look for specific problems

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17
Q

newborn presenting features

A

tachypnoea respiratory distress feeding issues - e.g. can’t breathe and feed at the same time

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18
Q

childhood presenting features

A

recurrent symptoms stridor/wheeze (wheeze is usually monophonic) recurrent pneumonia (structural abnormality predisposes them) cough feeding issues

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19
Q

how would CPAM appear on fetal imaging

A

white as it is often a cystic abnormality

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20
Q

tracheal agenesis

A

very rare presents at birth with acute respiratory distress and inability to intubate usually diagnosed before birth

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21
Q

tracheal stenosis

A

very rare complete cartilage rings - may be generalised or segmental present at birth or within first year 3 types, increasing narrowing of trachea, funnelling can create blockages

22
Q

tracheomalacia

A

more common floppiness of the airway dynamic abnormal collapse of tracheal walls closes readily during expiration, can be a problem during infection C shaped appearance in the back wall can be isolated in healthy infants

23
Q

what is tracheomalacia caused by

A

associated with genetic conditions may be caused by external compression e.g. vessels, tumour

24
Q

tracheomalacia presentation

A

barking cough recurrent croup SOB on exertion stridor/wheeze noisy in their regular breathing

25
Q

tracheomalacia manageent

A

physio and antibiotics when unwell natural history resolution with time SALBUTAMOL MAKES TRACHEOMALACIA WORSE

26
Q

tracheo-oesophageal fistula

A

abnormal connection between trachea and oesophagus association with genetic conditions primarily a GI problem but does affect both systems EA with distal TEF is most common

27
Q

how can tracheo-oesophageal fistula be diagnosed

A

antenatally (lots of fluid around baby) postnatally

28
Q

tracheo-oesophageal fistula presentation

A

choking colour change cough w/ feeding unable to pass NG tube

29
Q

tracheo-oesophageal treatment

A

surgical repair end to end anastamosis sometimes elongation is required depending on the severity of the condition

30
Q

tracheo-oesophageal fistula complications

A

tracheomalacia strictures leak reflux complications can still occur following repair

31
Q

congential pulmonary airway malformation (CPAM)

A

slightly more common abnormal non-functioning lung tissue can be cystic, vascular or trapped air in the non-functioning area 80% detected antenatally occur sporadically

32
Q

CPAM management

A

may resolve spontaneously in utero conservative management if asymptomatic surgical intervention may be required if there are respiratory difficulties possible risk of malignant change

33
Q

diaphragm development

A

essential for respiration develops from multiple tissues around 7wks innervated by phrenic nerve closure by ~18wks

34
Q

congenital diaphragmatic hernia

A

failure of closure 1/2500 births most common type is Bochdalek (90%) usually L side > R side organs from abdominal cavity move up into the chest compress lungs and heart, lung can remain small depending on the stage of development

35
Q

how are congenital diaphragmatic hernias diagnosed

A

mostly antenatally some cases are diagnosed later

36
Q

how are congenital diaphragmatic hernias managed

A

surgical repair prognosis depends on the degree of lung hypoplasia and the side affected

37
Q

eventration of diaphragm

A

incidental finding diaphragm is formed but is a bit thinner pulling up occurs unequal on either side

38
Q

changes in the lungs after birth

A

lungs inflate fluid in the lungs is absorbed

39
Q

transient tachypnoea of newborn

A

associated w/ C section improves with 1-2 days lungs aren’t squeezed s they come out the birth canal fluid isn’t absorbed as quickly and persists for a couple of days wet patchy appearance on the CXR indicates fluid

40
Q

what is surfactant made up from

A

complx mix of phospholipids and lipophillic proteins

41
Q

when do type II pneumocytes differentiate

A

24-24wks

42
Q

neonatal lung disease

A

24-34 wks but can happen in any pre-term infant up to 37-38 wks respiratory distress syndrome occurs in preterm infants with surfactant deficiency also called hyaline membrane disease

underexpanded lungs on CXR, patchy apperance

43
Q

treatment for neonatal lung disease

A

antenatal steroids to help mature baby’s lungs surfactant replacement appropriate ventilation and nutrition

44
Q

complications of neonatal lung disease

A

chronic lung disease associated w/ prematurity where ongoing oxygen requirement at term also called bronchopulmonary dysplasia multifactorial causes - lungs being ventilated w/ too much pressure, sepsis etc associated with increased childhood respiraoty morbidity future COPD?

45
Q

relationship between fetal/paediatric and adult lung disease

A

antenatal - nicotine exposure, infection, maternal nutrition (micronutrients and vitamins), low birth weight, prematurity, antenatal steroids post-natal - Barker hypothesis, infection, growth during childhood (corresponds w/ lung function), tobacco exposure, environemental pollution, micronutrients/vitamins

46
Q

what is remodelling?

A

the airway doesn’t stay static from birth but changes over time alteration of airway structure following external influence causes interference of inter-cellular signalling can perpetuate a cycle of chronic inflammation which damages the airway

47
Q

external influences on airway structure

A

environmental exposures chronic diseases of childhood infection

48
Q

remodelling - asthma

A

chronic inflammation increased bronchial responsiveness increase mucus secretion airway oedema airway narrowing

49
Q

remodelling - chronic lung disease

A

chronic inflammation interference in inter-cellular signalling treatment toxicity

50
Q

impact of early lung function into adulthood

A

lung function at birth and during childhood affects future resp health children born prematurely, peak at the same age but at a lower level then lung function decreases with age