Lung Development and Congenital Lung Disease

Question 1

intrauterine lung function

Answer 1

source of amniotic fluid

15 ml /Kg BW

Question 2

Affected by physical factors

Answer 2

Lung growth

Question 3

Affected by hormonal factors

Answer 3

Lung maturation

Question 4

Type 2 pneumocytes appear during

Answer 4

24-26 weeks

Question 5

Stimulates Maturation

Answer 5

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

Question 6

Inhibits Maturation

Answer 6

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

Question 7

Formation of the airways begins at

Answer 7

4 weeks AOG

Question 8

Differentiation begins at

Answer 8

16th week

Question 9

Surfactant synthesis begins at

Answer 9

24-26 weeks

Question 10

Embryonic phase

Answer 10

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

Question 11

Pseudoglandular phase

Answer 11

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

Question 12

Canalicular phase

Answer 12

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

Question 13

Terminal Saccular phase

Answer 13

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

Question 14

Alveolar Phase

Answer 14

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

Question 15

Characteristics of a mature alveolus

Answer 15

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

Question 16

Two stages of post natal lung growth

Answer 16

Increase in number until 2-8 years old

Increaselumen and size

Question 17

Pulmonary agenesis

Tracheal or laryngeal agenesis or stenosis

Answer 17

EMBRYONIC

Question 18

bronchial malformation
Ectopic lobes
AV malformation
Congenital lobar cysts

Answer 18

Tracheo or broncho-malacia

Question 19

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

Answer 19

Pseudoglandular

Question 20

Lung hypoplasia
Respiratory distress syndrome
Acinar dysplasia

Answer 20

Canalicular

Question 21

Pulmonary hypoplasia
RDS
Acinar dysplasia
Alveolar capillary dysplasia

Answer 21

Saccular/alveolar

Question 22

What syndrome is associated with Laryngeal or tracheal atresia

Answer 22

FRASER syndrome

Question 23

Most common congenital anomaly of the upper airway

Answer 23

Laryngomalacia

Question 24

Laryngomalacia

Answer 24

Dynamic anomaly of the supraglottis

Omega shape

Question 25

Unilateral VCP

Answer 25

Peripheral nerve L>R Aspiration, Coughing, choking

Question 26

Bilateral VCP

Answer 26

CNS High pitched inspiratory stridor Phonatory sound Inspiratory cry

Question 27

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

Answer 27

Subglottic hemangioma

Question 28

Diagnosis for Subglottic hemangioma

Answer 28

Endoscopy ; assymetric compressble bluish mass

Question 29

Bilateral Pulmonary Agenesis

Answer 29

Rare malformation | May occur in anencephaly

Question 30

Unilateral Pulmonary Agenesis

Answer 30

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

Question 31

Pulmonary apalsia

Answer 31

``` Absent lung Most common variant Unilateral Infections Absence of distal lung ```

Question 32

Causes of bilateral congenital small lungs

Answer 32

Lack of space Abnormal vascular supply Neuromuscular disease

Question 33

Causes of bilateral congenital small lungs due to extrapulmonary mechanical factors

Answer 33

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

Question 34

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

Answer 34

Foregut (Bronchogenic cysts)

Question 35

Cystic CTM | Spectrum of vasriably sized cysts with differing histology

Answer 35

congenital cystic adenomatoid malformation

Question 36

Stocker Classification: Incompatible with life

Answer 36

TYPE O Acinar dysplasia bronchial

Question 37

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

Answer 37

TYPE 1 Bronchial or bronchiolar

Question 38

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

Answer 38

Type 2 Bronchiolar

Question 39

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

Answer 39

Type 3 Bronchiolar or alveolar duct

Question 40

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

Answer 40

Type 4 Peripheral

Question 41

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

Answer 41

Pulmonary sequestration | -pulmanry tissue is cystic

Question 42

treatment for pulmonary sequestration

Answer 42

surgery

Question 43

Intrapulmonary sequestration

Answer 43

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

Question 44

Extrapulmonary sequestration

Answer 44

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

Question 45

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

Answer 45

COngenital large hypolucent lobe (congenital lobar exphysema) Most common : Left upper lobe 42%

Question 46

Incomplete mesodermal separation of the primitive foregut

Answer 46

Tacheoesophageal Fistula/esophageal atresia

Question 47

plain radiograph of TEF/EA

Answer 47

tube coiled in the upper pouch

Question 48

Main cause of death in isolated lesions of CDH

Answer 48

Pulmonary hypoplasia

Question 49

CDH that is more difficult to diagnose

Answer 49

Right sided

Question 50

cystic structure in chest, absence of intraabdominal stomach

Answer 50

Left sided CDH