Developmental aspects of lung disease

Question 1

Disease which can develop during the embryonic- post-glandular stage?

Answer 1

Embryonic/post-glandular

• airway stenosis-narrowing
• airway malaria-softening of tissue
• pulmonar hypoplasia/agenesis- incomplete development of the organ
• Tracheo-oesophageal fistula-abnormal connection

Question 2

How to detect lung development abnormalities

Answer 2

-fetal ultrasound (75%)
-newborn (10% ):
tachypnea
-childhood (15%):
wheeze/stridor
recurrent pneumonia
incidental finding

Question 3

Surfactant:

• what it is
• abnormalities

Answer 3

-Detergent which contains phospholipids and lipophilic proteins
-Surfactant proteins tend to develop around week 12-14
Abnormalities:
HYALINE MEMBRANE DISTRESS SYNDROME
treat with antenatal corticosteroids
Replace surfactant
Should have endogenous production of surfactant after day 5

Question 4

Fetal origins of COPD

Answer 4

ante-natal:
-nicotine 
-maternal micronutrients 
-fetal infection
-premature/low birth weight
Post-natal:
-infection 
-growth
-environmental pollution 
-micronutrients

Question 5

Remodelling

Answer 5

Change to structure following external influence
Pre-natal nicotine exposure:
-lung hypoplasia
-reduced alveolirisation
-reduced lung function; increased susceptibility to infection, increased incidence of wheeze

Question 6

Which diseases develop during the canalicular, saccular and alveolar stage?

Answer 6

-Bronchogenic cyst
-congenital pulmonary abnormalities:
Lobar emphysema
Sequestration
Congenital cyst adenomaloid malformation

Question 7

What is congenital cyst adenomaloid malformation?

Answer 7

Development of cysts of air or pleural fluid instead of proper lung tissue

Question 8

What is lung sequestration

Answer 8

When a section of lung tissue is not supplied by pulmonary circulation but by systemic

Question 9

Presenting features of congenital disease

Answer 9

Fetus: ultrasound
Newborn: Tachypnea and R.D.S.
Childhood: 
stridor/wheeze
Recurrent pneumonia 
Incidental findings

Question 10

Tracheo-broncho malacia

Answer 10

symptoms:
-barking coup
-S.O.B on exertion 
-Recurrent croup 
-stridor/wheeze 
Management:
-airway cleaning physiotherapy 
-antibiotics 
-AVOID ASTHMA MANAGEMENT 
should resolve spontaneously

Question 11

Pulmonary Adenomatoid malformation

Answer 11

cysts develop instead of lung tissue

Should resolve in Utero

Question 12

Diaphragmatic hernia

Answer 12

Two types:

• diaphragmatic: related to incomplete development of the diaphragm or weakness of the wall
• Hiatal: related to the hiatus in the diaphragm: stomach pushes up into chest

Question 13

Diaphragm eventration

Answer 13

Abnormal contouring of the diaphragm dome

Question 14

Functional diseases

Answer 14

Changes from Fluid secretion to absorption

• Tachypnea
• Resolves 24-48 hours
• associated with caesarian delivery

Question 15

Chronic neonatal lung disease

Answer 15

Also know as bronchopulmonary dysplasia/ CLD prematurity
Aetiology:
-antenatal infection
-genetic inheritance
-02 toxicity 
-PDA

Question 16

How can CNLD present in adulthood

Answer 16

Remodelling:

• chronic inflammation
• treatment toxicity
• interferance in inter-cellular signalling

Question 17

Remodelling in asthma

Answer 17

Asthma:

• increased mucus secretion
• airway oedema
• airway narrowing
• increased bronchial responsiveness.

Question 18

Fletcher- Peto curve

Answer 18

Shows how disease can present itself over the years
Respiratory symptoms tend to show up at 20 years
-Normal
-rapid decline
-early decline
-reduced growth