Developmental Aspects of Lung Disease

Question 1

In what stage of morphogenesis does surfactant being to develop

Answer 1

Saccular stage

Question 2

Common upper congenital abnormalities

Answer 2

Tracheal anagenesis and stenosis
Tracheomalacia
Tracheo-oesphageal fistula

Question 3

Common lower congenital abnormalities

Answer 3

Lung anagenesis/pulmonary hypoplasia
Bronchogenic cyst
Congenital pulmonary airway malformation (CPAM)
Congenital diaphragmatic hernia

Question 4

Presenting features in antenatal scanning

Answer 4

Ultrasound - show up more at 20 weeks scan

Question 5

Presenting features in newborns

Answer 5

Tachypnoea
Respiratory distress
Feeding issues

Question 6

Tachypnoea

Answer 6

Abnormally rapid breathing

Question 7

Presenting features in childhood

Answer 7

Stridor/wheeze
Recurrent pneumonia
Cough
Feeding issues

Question 8

Asymptomatic presenting features

Answer 8

Incidental finding

Question 9

Tracheal agenesis

Answer 9

Trachea is absent or underdeveloped

Question 10

Presentation of tracheal agenesis

Answer 10

Acute respiratory distress at birth

Question 11

Tracheal stenosis

Answer 11

Tracheal cartilage rings are completed too early, may be generalised or segmental

Question 12

Time of detection of tracheal stenosis

Answer 12

At birth or within first year

Question 13

Tracheomalacia

Answer 13

Dynamic abnormal collapse of tracheal walls

Question 14

Presentation of tracheomalacia

Answer 14

Braking cough
Recurrent croup
Breathless on exertion
Stridor/wheeze

Question 15

Management of tracheomalacia

Answer 15

Physiotherapy and antibiotics when unwell, natural resolution with time

Question 16

Causes of tracheomalacia

Answer 16

Genetic conditions

External compression

Question 17

Tracheo-oesphageal fistula

Answer 17

Abnormal connection between trachea and oesophagus

Question 18

Presentation of tracheo-oesphageal fistula

Answer 18

Choking
Colour change
Cough with feeding
Unable to pass nasal-gastric tube

Question 19

Treatment of trachea-oesphageal fistula

Answer 19

Surgical repair (rejoin or elongation)

Question 20

Complications of trachea-oestophageal fistula

Answer 20

Tracheomalacia
Strictures
Leak
Reflux

Question 21

Time of detection of trachea-oesphageal fistula

Answer 21

Antenatal or postnatal

Question 22

Congenital pulmonary airway malformation (CPAM)

Answer 22

Abnormal non-functioning lung tissue, occurs sporadically

Question 23

Management of CPAM

Answer 23

May resolve spontaneously in utero
Conservative treatment id asymptomatic
Surgical intervention

Question 24

Time of detection of CPAM

Answer 24

80% detected antenatally

Question 25

Diaphragm development

Answer 25

Develops from multiple tissue at 7 weeks and closes by 18 weeks

Question 26

Cause of congenital diaphragmatic hernia

Answer 26

Failure of closure of diaphragm (usually left side)

Question 27

Time of diagnosis of congenital diaphragmatic hernia

Answer 27

Most antenatally, some cases late

Question 28

Management of congenital diaphragmatic hernia

Answer 28

Surgical repair - primary closure

Question 29

Eventration of diaphragm

Answer 29

Abnormal elevation of dome of diaphragm in which all or part of it is largely composed of fibrous tissue

Question 30

Change in lungs after birth

Answer 30

Lungs inflate and fluid in lungs is absorbed

Question 31

Transient tachypnoea of newborn

Answer 31

Accumulation of fluid in lungs due to no squeezing of lungs after birth

Question 32

At which weeks do type II pneumocytes differentiate

Answer 32

Weeks 24-34

Question 33

What is surfactant made up of

Answer 33

Phospholipids and lipophilic proteins

Question 34

Other name of RDS

Answer 34

Hyaline membrane disease

Question 35

What causes RDS

Answer 35

Surfactant deficiency

Question 36

Treatment of RDS

Answer 36

Antenatal steroids (matures lungs) Surfactant replacement Appropriate ventilation and nutrition

Question 37

Bronchopulmonary dysplasia

Answer 37

Chronic lung disease that results from damage to lungs caused by mechanical ventilation and long term use of oxygen

Question 38

Remodelling

Answer 38

Alteration of airway structure following external influence

Question 39

Result of remodelling

Answer 39

Interference of inter-cellular signalling

Question 40

Causes of remodelling

Answer 40

``` Environmental influences Chronic diseases in childhood Infection Gene interactions Antenatal programming Early allergy ```

Question 41

Remodelling in asthma

Answer 41

``` Chronic inflammation Increased bronchial responsiveness Increase mucus secretion Airway oedema Airway narrowing ```

Question 42

Remodelling in chronic lung disease

Answer 42

Chronic inflammation Interference of inter-cellular signalling Treatment toxicity - can cause structural change in airways

Question 43

Pros of prediction of future lung development

Answer 43

Allows identification of illnesses that will appear later in life and so allows for earlier treatment