cystic fibrosis, surgery & pulmonary vascular disease

Question 1

what type of genetic disorder is cystic fibrosis?

Answer 1

autosomal recessive disorder

Question 2

what is cystic fibrosis?

Answer 2

pulmonary disease which causes morbidity and mortality

leads to respiratory failure death in 90% of patients

Question 3

what causes cystic fibrosis?

Answer 3

mutation in the CFTR protein causes abnormal transport of Na and Cl causes impaired bacteria killing and dehydrated mucous layer which creates thick sticky mucous

Question 4

what are the different classes of cystic fibrosis?

Answer 4

I-III, severe

IV-VI, milder

Question 5

what are the different screening tests for cystic fibrosis?

Answer 5

antenatal- pre implantation genetic diagnosis, chorionic villous sampling, amniocentesis
postnatal- guthie test on day 5 and sweat test

Question 6

what would a CT show if cystic fibrosis was present?

Answer 6

tramlines, signet rings, mucous plugging and consolidation

Question 7

how would you manage cystic fibrosis in adults?

Answer 7

replace enzymes (creon)
high energy diet + high calorie supplement drinks
nutritional supplements

Question 8

in cystic fibrosis how would mucous obstruction inflammation be treated?

Answer 8

physiotherapy
mucolytics
bronchodilators

Question 9

in cystic fibrosis how would chronic infection be treated?

Answer 9

oral/IV/nebulised antibiotics

Question 10

in cystic fibrosis how would inflammation be treated?

Answer 10

azithromycin

Question 11

in cystic fibrosis how would fibrosis be treated ?

Answer 11

supportive management because it is irreversible

Question 12

what are some common complications of cystic fibrosis ?

Answer 12

pancreatic insufficiency, recurrent chest infections, diabetes, osteoporosis, pneumothorax, haemoptysis

Question 13

which classes of cystic fibrosis lead to pancreatic insufficiency?

Answer 13

I-III

Question 14

what are the complications of pancreatic insufficiency?

Answer 14

malabsorption
abnormal stool
failure to thrive

Question 15

when cystic fibrosis patients present with recurrent chest infections what do their survival depend on?

Answer 15

FEV1

Question 16

what are the complications of recurrent chest infections?

Answer 16

pneumonia, brochiectasis, scarring, growing accesses and progressive airflow obstruction

Question 17

when would a cystic fibrosis patient be eligible for a lung transplant?

Answer 17

rapidly deteriorating lung function
FEV1 <30% predicted
life threatening exacerbations
estimated survival less than 2 years

Question 18

what procedure is performed to fix a pneumothorax?

Answer 18

pleurodesis

Question 19

what procedures are performed to fix empyema?

Answer 19

pleurectomy and decortication

Question 20

what are bronchogenic cysts?

Answer 20

they are lined by bronchial mucosa
present from birth
press on trachea or oesophagus and cause dysphagia and stridor

Question 21

describe a pulmonary embolism?

Answer 21

thrombus forms in venous system and embolisms to the pulmonary arteries

Question 22

what are risk factors for a pulmonary embolism?

Answer 22

major trauma, surgery, cancer, cardiopulmonary disease, pregnancy or inherited thrombophilia

Question 23

what are symptoms for a venous thromboembolism?

Answer 23

pleuritic chest pain, cough and haemoptysis
isolated acute dyspnoea
syncope, cardiac arrest

Question 24

what are symptoms for pulmonary embolism?

Answer 24

pyrexia, pleural rub and pleural effusion

tachycardia, tachypnoea and hypoxia

Question 25

what are the 2 scoring systems used to diagnose a pulmonary embolism?

Answer 25

wells score and revised Geneva score

Question 26

what investigations can be done to diagnose a pulmonary embolism?

Answer 26

various blood tests
chest xray
ECG
CT pulmonary angiogram
V/Q scan
echocardiography
D-dimer (blood clotting test)

Question 27

whats the treatment for a pulmonary embolism?

Answer 27

oxygen
heparin/warfarin
direct oral anticoagulants 
thrombolysis
pulmonary embolectomy

Question 28

what are the causes for pulmonary hypotension ?

Answer 28

idiopathic
secondary to left heart disease
secondary to chronic respiratory disease
chronic thromboembolic pulmonary hypotension

Question 29

what is chronic thromboembolic pulmonary hypotension?

Answer 29

unresolved pulmonary embolism where patient has developed chronic remodelling of pulmonary arterial tree which constricts blood vessels

Question 30

what are physical symptoms of pulmonary hypotension?

Answer 30

exertion dyspnoea
chest tightness
exertion syncope
hepatomegaly
ankle oedema 
haemoptysis (rarely)

Question 31

what clinical symptoms may present in pulmonary hypotension?

Answer 31

elevated jugular venous pressure
right ventricular heave
loud pulmonary second heart sound

Question 32

what investigations are carried out to diagnose pulmonary hypotension?

Answer 32

ECG
lung function test
chest xray
echocardiograph
V/Q scan
CTPA (CT pulmonary angiogram)
right heart catheterisation

Question 33

whats the first line treatment for pulmonary hypotension?

Answer 33

endothelin receptor antagonist

Question 34

what are other treatments for pulmonary hypotension ?

Answer 34

treat underlying condition
oxygen
anticoagulation
diuretics

Question 35

when are diuretics used?

Answer 35

when patients get peripheral oedema