UWORld Flashcards

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1
Q

What kind of channel is the CFTR channel?

A

Transmembrane ATP-gated chloride channel

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2
Q

What test is done to screen for CF?

A

Sweat chloride test

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3
Q

What is congenital long QT syndrome due to?

A

Mutation in voltage gated K+ channel

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4
Q

What are the clinical signs of epiglottitis?

A
  • Distress (tripod position, sniffing position, stridor)
  • Dysphagia, dysphonia
  • Drooling
  • High fever
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5
Q

Does anaphylaxis cause fever?

A

No

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6
Q

What does alpha1 antitrypsin inhibit in the lung?

A

Neutrophil elastase

-> Less elastin broken down

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7
Q

What virus most commonly causes croup?

A

Paramyxovirus

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8
Q

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

A

Activates medullary sigma receptors

- Cough suppresant

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9
Q

What is the most common cause of primary lung cancer?

A

Adenocarcinoma

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10
Q

What are the levels of vasopressin in septic shock?

A

Suppressed

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11
Q

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

A

Lactated Ringer solution

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12
Q

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

A
  • Exudative phase
  • Proliferative phase
  • Fibrotic phase
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13
Q

How can ARDS be identified?

A

Bilateral pulmonary infiltrates and hypoxemia in the absence of HF

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14
Q

What is a characteristic sign of pulmonary fibrosis on CT?

A

Honeycombing - dilation of terminal bronchioles due to fibrosis

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15
Q

What is elastase contained in/by?

A
  • Macrophage lysosomes

- Azurophilic (primary) granules of neutrophils

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16
Q

What is macrophage elastase inhibited by?

A

Tissue inhibitors of metalloproteinasis

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17
Q

How may alpha1 antitrypsin deficiency cause liver cirrhosis?

A

Misfolded alpha1 antitrypsin aggregates in the hepatocytes

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18
Q

What does periodic acid schiff stain glycoproteins such as A1AT?

A

pink

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19
Q

Is AIAT diatase resistant?

A

Yes

20
Q

What is lactulose used for in liver disease?

A

To prevent hepatic encaphalopathy

21
Q

What causes desmosine crosslinks?

A

Lysyl oxidase

22
Q

What is the most common mutation of CFTR?

A

delta F508 mutation

- Deletion

23
Q

How does deltaF508 mutation causes CF?

A
  • Abnormal protein folding
  • Channel is degraded before reaching surface
  • Also reduces channel opening
  • ATP unable to bind
24
Q

What is the mutation which causes a1AT deficiency?

A

SERPINA1

- PiZ mutation causes a misfold

25
Q

What is the normal A1AT?

A

PiM

26
Q

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

A
  • Pan-acinar emphysema
  • Chronic bronchitis
  • Bronchiectasis
27
Q

Is elastin hydrophobic or hydrophilic?

A

Hydrophobic, mostly non-polar

28
Q

How does surfactant affect the lungs?

A
  • Increases compliance (opening of lungs)
  • Decreased surface tension (ability to open)
  • Decreased elastic recoil (ability for alveoli to close)
  • Decreased alveolar collapse (stay open)
29
Q

What is methacholine used for?

A

Bronchoprovocation test

- Diagnosis of asthma

30
Q

What kind of drug is methacholine?

A

Non-selective muscarinic receptor agonist

31
Q

What may cause ARDS?

A
  • Pulmonary trauma - contrusions, irritants

- Indirect nonpulmonary insults - sepsis, pancreatitis, burns

32
Q

What are symptoms of granulomatosis w. polyangitis?

A
  • Cough w. hemoptysis
  • Nephritic syndrome
  • Necrotizing inflammation + pulmonary hemorrhage
33
Q

What are the functions of club/clara cells?

A
  • Degrade toxins via surfactant w. CYP450
  • Secrete secretoglobin famiy 1A member 1
  • Protects the bronchiolar epithelium
  • It is a bronchiolar exocrine cell
34
Q

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

A

Centracinar emphysema = Smoking

Panacinar emphysema = A1AT def

35
Q

What are the ABG values in PE?

A
  • Increased pH
  • Normal HCO3-
  • Decreased O2 and CO2
36
Q

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

A

Contained in T2 pneumocytes

- Store and release surfactant

37
Q

What does intrapulmonary shunting mean?

A

Adequetly perfused but poorly ventilated

38
Q

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

A

< 1.5

> 2 indicates maturity

39
Q

What are the risk factors for NRDS?

A
  • Prematurity
  • Maternal diabetes
  • C-section
40
Q

What are the treatments for NRDS?

A
  • Decreased release of fetal glucocorticoids

- Exogenous surfactant for infant

41
Q

What can therapeutic supplemental O2 rsult in?

A
  • Retinopathy of prematurity
  • Intraventricular hemorrhage
  • Bronchopulmonary dysplasia
42
Q

What do alveolar macrophages releasse?

A
  • Cytokines

- Alveolar proteases

43
Q

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

A

Hemosiderin-laden macrophages (HF cells)

44
Q

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

A

basophilic purple/blue appearence on LM

45
Q

What granules are released in actinomycosis?

A

Sulfur (formed by calcified mycelial fragments)

46
Q

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

A

Aspiration

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

47
Q

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

A

Restrictive pattern

  • Decreased FEV1 and FVC
  • Decreased ERV and RV
  • Decreased TLC
  • Normal Residual Volume