UWORld

Question 1

What kind of channel is the CFTR channel?

Answer 1

Transmembrane ATP-gated chloride channel

Question 2

What test is done to screen for CF?

Answer 2

Sweat chloride test

Question 3

What is congenital long QT syndrome due to?

Answer 3

Mutation in voltage gated K+ channel

Question 4

What are the clinical signs of epiglottitis?

Answer 4

• Distress (tripod position, sniffing position, stridor)
• Dysphagia, dysphonia
• Drooling
• High fever

Question 5

Does anaphylaxis cause fever?

Answer 5

No

Question 6

What does alpha1 antitrypsin inhibit in the lung?

Answer 6

Neutrophil elastase

-> Less elastin broken down

Question 7

What virus most commonly causes croup?

Answer 7

Paramyxovirus

Question 8

What receptors does dextromorphan act on and what are its effects/

Answer 8

Activates medullary sigma receptors

- Cough suppresant

Question 9

What is the most common cause of primary lung cancer?

Answer 9

Adenocarcinoma

Question 10

What are the levels of vasopressin in septic shock?

Answer 10

Suppressed

Question 11

What fluid may be used in septic shock, outside of 0.9% NaCl?

Answer 11

Lactated Ringer solution

Question 12

What are the 3 phases of Acute Respiratory Distress Syndrome (ARDS)?

Answer 12

• Exudative phase
• Proliferative phase
• Fibrotic phase

Question 13

How can ARDS be identified?

Answer 13

Bilateral pulmonary infiltrates and hypoxemia in the absence of HF

Question 14

What is a characteristic sign of pulmonary fibrosis on CT?

Answer 14

Honeycombing - dilation of terminal bronchioles due to fibrosis

Question 15

What is elastase contained in/by?

Answer 15

• Macrophage lysosomes

- Azurophilic (primary) granules of neutrophils

Question 16

What is macrophage elastase inhibited by?

Answer 16

Tissue inhibitors of metalloproteinasis

Question 17

How may alpha1 antitrypsin deficiency cause liver cirrhosis?

Answer 17

Misfolded alpha1 antitrypsin aggregates in the hepatocytes

Question 18

What does periodic acid schiff stain glycoproteins such as A1AT?

Answer 18

pink

Question 19

Is AIAT diatase resistant?

Answer 19

Yes

Question 20

What is lactulose used for in liver disease?

Answer 20

To prevent hepatic encaphalopathy

Question 21

What causes desmosine crosslinks?

Answer 21

Lysyl oxidase

Question 22

What is the most common mutation of CFTR?

Answer 22

delta F508 mutation

- Deletion

Question 23

How does deltaF508 mutation causes CF?

Answer 23

• Abnormal protein folding
• Channel is degraded before reaching surface
• Also reduces channel opening
• ATP unable to bind

Question 24

What is the mutation which causes a1AT deficiency?

Answer 24

SERPINA1

- PiZ mutation causes a misfold

Question 25

What is the normal A1AT?

Answer 25

PiM

Question 26

What 3 conditions in the lungs can a1AT deficiency cause in the lungs?

Answer 26

- Pan-acinar emphysema - Chronic bronchitis - Bronchiectasis

Question 27

Is elastin hydrophobic or hydrophilic?

Answer 27

Hydrophobic, mostly non-polar

Question 28

How does surfactant affect the lungs?

Answer 28

- Increases compliance (opening of lungs) - Decreased surface tension (ability to open) - Decreased elastic recoil (ability for alveoli to close) - Decreased alveolar collapse (stay open)

Question 29

What is methacholine used for?

Answer 29

Bronchoprovocation test | - Diagnosis of asthma

Question 30

What kind of drug is methacholine?

Answer 30

Non-selective muscarinic receptor agonist

Question 31

What may cause ARDS?

Answer 31

- Pulmonary trauma - contrusions, irritants | - Indirect nonpulmonary insults - sepsis, pancreatitis, burns

Question 32

What are symptoms of granulomatosis w. polyangitis?

Answer 32

- Cough w. hemoptysis - Nephritic syndrome - Necrotizing inflammation + pulmonary hemorrhage

Question 33

What are the functions of club/clara cells?

Answer 33

- Degrade toxins via surfactant w. CYP450 - Secrete secretoglobin famiy 1A member 1 - Protects the bronchiolar epithelium - It is a bronchiolar exocrine cell

Question 34

What type of emphysema affects smokers vs what type of emphysema affects those with A1AT deficiency?

Answer 34

Centracinar emphysema = Smoking Panacinar emphysema = A1AT def

Question 35

What are the ABG values in PE?

Answer 35

- Increased pH - Normal HCO3- - Decreased O2 and CO2

Question 36

What do the lamellar bodies do and what cells are they contained in?

Answer 36

Contained in T2 pneumocytes | - Store and release surfactant

Question 37

What does intrapulmonary shunting mean?

Answer 37

Adequetly perfused but poorly ventilated

Question 38

A Lecithin/sphingomyelin (L:S) ratio below what vaule indicates Neonatal Respiratory Distress Syndrome?

Answer 38

< 1.5 > 2 indicates maturity

Question 39

What are the risk factors for NRDS?

Answer 39

- Prematurity - Maternal diabetes - C-section

Question 40

What are the treatments for NRDS?

Answer 40

- Decreased release of fetal glucocorticoids | - Exogenous surfactant for infant

Question 41

What can therapeutic supplemental O2 rsult in?

Answer 41

- Retinopathy of prematurity - Intraventricular hemorrhage - Bronchopulmonary dysplasia

Question 42

What do alveolar macrophages releasse?

Answer 42

- Cytokines | - Alveolar proteases

Question 43

What cells may be found in the setting of pulmonary oedema or alveolar hemorhage?

Answer 43

Hemosiderin-laden macrophages (HF cells)

Question 44

What will the effects of the HandE stain be on actinomycosis?

Answer 44

basophilic purple/blue appearence on LM

Question 45

What granules are released in actinomycosis?

Answer 45

Sulfur (formed by calcified mycelial fragments)

Question 46

What is a major risk factor for actinomycosis infection of the lungs?

Answer 46

Aspiration | - Most commonly found in mouth and may result in cervicofacial abscesses

Question 47

How does obesity-related restrictive lung disease affect pulmonary function test results?

Answer 47

Restrictive pattern - Decreased FEV1 and FVC - Decreased ERV and RV - Decreased TLC - Normal Residual Volume