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OS 213 Respiratory EXAM 1 > Lung Development and Congenital Lung Disease > Flashcards

Lung Development and Congenital Lung Disease Flashcards

1
Q

intrauterine lung function

A

source of amniotic fluid

15 ml /Kg BW

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2
Q

Affected by physical factors

A

Lung growth

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3
Q

Affected by hormonal factors

A

Lung maturation

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4
Q

Type 2 pneumocytes appear during

A

24-26 weeks

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5
Q

Stimulates Maturation

A 
glucocorticoids, ACTH
Thyroid hormones, TR
EGF
heroin
Aminophyline, cAMP
Interferon
Estrogens
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6
Q

Inhibits Maturation

A 
Diabetes (insuin,hyperglycemia, butyric acid)
Testosterone
TGF-B
Barbiturates
Prolactin
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7
Q

Formation of the airways begins at

A

4 weeks AOG

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8
Q

Differentiation begins at

A

16th week

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9
Q

Surfactant synthesis begins at

A

24-26 weeks

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10
Q

Embryonic phase

A 
3-6 weeks
Laryngotracheal groove
Fibroblast growth factor
tracheoesophageal septum
tracheal bud
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11
Q

Pseudoglandular phase

A

6-16 weeks
resembles endocrine gland
all major lung elements have appeared
Respiration is not possible during this phase

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12
Q

Canalicular phase

A

16-26 weeks
Lumina of bronchi enlarge and lung tissue becomes highly vascularized
Respiration is possible

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13
Q

Terminal Saccular phase

A

26-36 weeks
blood air barrier is established
Type 1 and type 2

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14
Q

Alveolar Phase

A

Birth to 8 years old
True alveoli appear as indentations
Structurally and functionally well-differentiated

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15
Q

Characteristics of a mature alveolus

A
  1. connected to alveolar duct
  2. Lined with type 1 cells in intimate contact with cap
  3. Each capillary exposed to 2 alveoli
  4. contains surfactant
  5. Has interconnections with adjacent alveoli
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16
Q

Two stages of post natal lung growth

A

Increase in number until 2-8 years old

Increaselumen and size

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17
Q

Pulmonary agenesis

Tracheal or laryngeal agenesis or stenosis

A

EMBRYONIC

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18
Q

bronchial malformation
Ectopic lobes
AV malformation
Congenital lobar cysts

A

Tracheo or broncho-malacia

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19
Q 
Cystic adenomatoid malformatiion
Pulmonary sequestration
lung hypoplasia
lung cysts
congenital pulmonary lymphagiectasia
congenital diagphragmatic hernia
A

Pseudoglandular

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20
Q

Lung hypoplasia
Respiratory distress syndrome
Acinar dysplasia

A

Canalicular

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21
Q

Pulmonary hypoplasia
RDS
Acinar dysplasia
Alveolar capillary dysplasia

A

Saccular/alveolar

22
Q

What syndrome is associated with Laryngeal or tracheal atresia

A

FRASER syndrome

23
Q

Most common congenital anomaly of the upper airway

A

Laryngomalacia

24
Q

Laryngomalacia

A

Dynamic anomaly of the supraglottis

Omega shape

25
Q

Unilateral VCP

A

Peripheral nerve
L>R
Aspiration, Coughing, choking

26
Q

Bilateral VCP

A

CNS
High pitched inspiratory stridor
Phonatory sound
Inspiratory cry

27
Q

Benign vascular tumor with rapid growth phase for 12 to 18 months followed by involution.
L>R

A

Subglottic hemangioma

28
Q

Diagnosis for Subglottic hemangioma

A

Endoscopy ; assymetric compressble bluish mass

29
Q

Bilateral Pulmonary Agenesis

A

Rare malformation

May occur in anencephaly

30
Q

Unilateral Pulmonary Agenesis

A

More common
Absence of carina and trachea
Mortality R> L
Lung larger than normal

31
Q

Pulmonary apalsia

A 
Absent lung
Most common variant
Unilateral
Infections
Absence of distal lung
32
Q

Causes of bilateral congenital small lungs

A

Lack of space
Abnormal vascular supply
Neuromuscular disease

33
Q

Causes of bilateral congenital small lungs due to extrapulmonary mechanical factors

A

Abnormal thoracic contents
Thoracic compression from below
Thoracic compression from the sides

34
Q

Closed epithelium lined sacs developing abnormally, more common cysts in infancy
50% located in mediastinum close to carina
single unilocular R?L

A

Foregut (Bronchogenic cysts)

35
Q

Cystic CTM

Spectrum of vasriably sized cysts with differing histology

A

congenital cystic adenomatoid malformation

36
Q

Stocker Classification: Incompatible with life

A

TYPE O Acinar dysplasia bronchial

37
Q

Stocker classification: Most common, best prognosis, one part of one lobe, PSCCE, Multiloculated

A

TYPE 1 Bronchial or bronchiolar

38
Q

Stocker classification: 2nd most common, infection in later childhood, overgrowth of dilated bronchiolar structures separated by alveolar tissue

A

Type 2 Bronchiolar

39
Q

Stocker Classification: Male infants, Whole lobe, appear solid, Absent pulmonary arteries

A

Type 3 Bronchiolar or alveolar duct

40
Q

Stocker Classification: rare, peripheral thin-walled cysts, multiloculated, cystic spaces lined by alveolar type 1 or type 2 cells, intervening stroma are thin and comprise loose mesenchymal tissue

A

Type 4 Peripheral

41
Q

Pulmonary tissue that is isolated from normal functioning and is fed by systemic arteries

A

Pulmonary sequestration

-pulmanry tissue is cystic

42
Q

treatment for pulmonary sequestration

A

surgery

43
Q

Intrapulmonary sequestration

A

Posterior basal segment of left lower lobe
Encricled by viscera
Rest of lobe is normal
Systemic drainage

44
Q

Extrapulmonary sequestration

A 
Beneath the left lower lobe
15% abdominal
M>F
systemic arteries are small
Pulmonary drainage
45
Q

Deficiency of bronchial cartilage leading to ianpropriate collapse of the airway and trapping of the air

A

COngenital large hypolucent lobe (congenital lobar exphysema)

Most common : Left upper lobe 42%

46
Q

Incomplete mesodermal separation of the primitive foregut

A

Tacheoesophageal Fistula/esophageal atresia

47
Q

plain radiograph of TEF/EA

A

tube coiled in the upper pouch

48
Q

Main cause of death in isolated lesions of CDH

A

Pulmonary hypoplasia

49
Q

CDH that is more difficult to diagnose

A

Right sided

50
Q

cystic structure in chest, absence of intraabdominal stomach

A

Left sided CDH

OS 213 Respiratory EXAM 1 (19 decks)

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