Restrictive Disease and Developmental Aspects

Question 1

Interstitium definition

Answer 1

Connective tissue space around airways and vessels and space between basement membrane and alveolar walls

Question 2

Is inhalation or exhalation more difficult with restrictive lung disease?

Answer 2

Inspiration

Question 3

Effect of restrictive disease on FEV1, FVC, FEV1/FVC ratio

Answer 3

Low FEV1, low FVC (<80% of normal), ratio is normal

Question 4

Sarcoidosis definition

Answer 4

Multisystem granulomatous disorder of unknown aetiology

Question 5

Does sarcoidosis feature caseating or non-caseating granulomas?

Answer 5

Non-caseating

Question 6

Presentation of sarcoidosis

Answer 6

Acute arthalgia, erythema nodosum
SoB
Cough
Abnormal CXR

Question 7

Hypersensitivity pneumonitis antigens

Answer 7

Bird/animal proteins
Fungi
Chemicals
Thermophilic actinomycetes

Question 8

Presentation of acute hypersensitivity pneumonitis

Answer 8

Fever, dry cough, muscle aches
Chills
Crackles, wheeze

Question 9

Presentation of chronic hypersensitivity pneumonitis

Answer 9

Malaise, SoB, cough
Low grade illness
Crackles

Question 10

Where in lungs does usual interstitial pneumonitis primarily affect

Answer 10

Diffuse but mainly basal and posterior

Question 11

Values that define Type 1 and Type 2 respiratory failure

Answer 11

Type 1: PaO2<8kPa (PaCO2 normal or low)

Type 2: PaCO2>6.5kPa (PaO2 usually low)

Question 12

4 causes of hypoxaemia

Answer 12

Ventilation/perfusion mismatch
Diffusion impairment
Alveolar hypoventilation
Shunt

Question 13

Difference between apnoea and hypopnoea

Answer 13

Apnoea=complete blocking

Hypopnoea=partial blocking

Question 14

Direct cause of sleep apnoea

Answer 14

Upper airway obstruction

Question 15

Severity scoring system/questionnaire for sleep apnoea

Answer 15

Epworth Sleepiness Scale

Question 16

3 treatments for sleep apnoea

Answer 16

Find and treat exacerbating factors (eg obesity etc.)

Continuous positive Airways pressure (CPAP)(sleep apnoea mask)

Question 17

Narcolepsy definition

Answer 17

Overwhelming daytime drowsiness and sudden attacks of sleep

Question 18

Clinical features of narcolepsy

Answer 18

Cataplexy
Daytime tiredness
Hallucinations when waking up/falling asleep
Sleep paralysis

Question 19

Main investigation for sleep disorders

Answer 19

Polysomnography

Question 20

Treatments for narcolepsy

Answer 20

Modafinil (stimulant)
Dexamphetamine (stimulant)
Venlafaxine (for cataplexy)
Sodium oxybate

Question 21

Defining features for chronic ventilatory failure

Answer 21

Elevated pCO2 (>6kPa)
pO2 < 8kPa
Normal blood pH
Elevated bicarbonate

Question 22

Treatment for chronic ventilatory failure

Answer 22

Non-invasive ventilation

Oxygen therapy

Question 23

Laryngomalacia definition

Answer 23

Collapse/softening of larynx

Question 24

Presentation of laryngomalacia

Answer 24

Stridor

Worse when feeding or upset

Question 25

Traceomalacia definition

Answer 25

Softening of trachea

Question 26

Presentation of tracheomalacia

Answer 26

Barking cough
Recurrent croup
Breathlessness in exertion
Stridor/wheeze

Question 27

Presentation of tracheo-oesophageal fistula

Answer 27

Choking
Colour change
Cough with feeding
Unable to pass NG tube

Question 28

Stages of lung development

Answer 28

Embryonic: 3-8 weeks
Pseudoglandular: 5-17 weeks
Canalicular: 16-26 weeks
Saccular: 24-38 weeks
Alveolar: 36 weeks-2/3 years

Question 29

What happens during embryonic phase?

Answer 29

Lung buds start to develop

Question 30

What happens during pseudoglandular phase?

Answer 30

Rapid branching of airways

Question 31

What happens during canalicular phase?

Answer 31

Gas exchange units formed

Type 1 and 2 pneumocyte production

Question 32

What happens during saccular phase?

Answer 32

Alveolar sacs grow and become well formed

Question 33

What happens during alveolar phase?

Answer 33

Can independently support breathing