Tuesday TBL

Question 1

When should you consider alpha one antitrypsin deficiency?

Answer 1

Emphysema in a young individual (eg, age ≤45 years) ●Emphysema in a nonsmoker or minimal smoker ●Emphysema with predominant basilar lucency on the chest radiograph ●A family history of emphysema and/or liver disease ●Clinical findings or history of panniculitis ●Clinical findings or history of unexplained chronic liver disease

Question 2

What does a deficiency of alpha-1 antitrypsin result in?

Answer 2

development of early emphysema

Question 3

In COPD cases, which med can you never use alone? what do you have to couple it with?

Answer 3

can’t use inhaled steroids alone, must couple with LABA

Question 4

What’s the MOA of β-adrenergic agonists ?

Answer 4

working through Gs to increase ↑ cAMP levels, reducing myosin light chain phosphorylation (and hence contraction), and to open large-conductance calcium-activated potassium channels, hyperpolarizing airway smooth muscle, which further reduces contraction. —Both effects relax airway smooth muscle bronchodilation.

Question 5

Presence of permanently dilated, damaged major bronchi and bronchioles Response to inflammatory/infectious insult over time =

Answer 5

bronchiectasis this is a manifestation or complication of an underlying disease or condition. need to find cause

Question 6

What kind of disease is Kartagener syndrome?

Answer 6

primary ciliary dyskinesia

Question 7

Asthma has a delayed and an immediate response. What brings about the delayed response?

Answer 7

eosinophils and neutrophils are activated by interleukins and other mediators and go on to release additional mediators of bronchoconstriction

Question 8

Which asthma inhibitors are administered orally, making them beneficial for kiddos?

Answer 8

leukotriene pathway inhibitors

Question 9

Mutations in any of several genes encoding for proper structure and function of cilia, such as a dynein arm defect =

Answer 9

primary ciliary dyskinesia

Question 10

What’s the treatment for ABPA (Allergic Bronchopulmonary Aspergillosis)?

Answer 10

prednisone (b/c it’s an inflammatory process, not an infectious process)

Question 11

Airflow obstruction that is highly reversible/variable in response to medication or spontaneously= ?

Answer 11

asthma

Question 12

Asthma has a delayed and an immediate response. What brings about the immediate response?

Answer 12

allergens trigger mast cells and T lymphocytes to release, histamine, prostaglandins, and leukotrienes

Question 13

What’s the MOA of Omalizumab?

Answer 13

monoclonal anti-IgE antibody that binds to IgE and inhibits its binding to mast cells

Question 14

What triad is seen in Kartagener syndrome?

Answer 14

sinusitis bronchiectasis situs inversus

Question 15

What chromosome is the CFTR gene on?

Answer 15

chromosome 7

Question 16

What’s the MOA of corticosteroids?

Answer 16

Corticosteroids decrease the transcription of genes for many pro-inflammatory proteins and increase transcription of genes coding for several anti-inflammatory proteins and the β2-adrenergic receptor.

Question 17

How do you make a diagnosis of bronchiectasis?

Answer 17

chest imaging; high resolution CT is “gold standard”

Question 18

What are the most common LABAs?

Answer 18

salmeterol and formoterol

Question 19

What will you see on chest X-ray that points to bronchiectasis?

Answer 19

“tram tracks”: thickened bronchial walls seen longitudinally -and finger glove sign (mucus filled dilated bronchi)

Question 20

What are some common exam findings in CF patients?

Answer 20

-Clubbing is almost always seen -Nasal mucosal changes and polyps common -crackles on exam, not wheezes -underweight/malnutrition

Question 21

What’s the inheritance pattern of cystic fibrosis?

Answer 21

autosomal recessive

Question 22

What’s the most important drug class in the treatment of asthma?

Answer 22

inhaled corticosteroids

Question 23

What’s the methylxanthine we have to know?

Answer 23

theophylline

Question 24

What’s the MOA of methylxanthines?

Answer 24

inhibit phospodiesterases =increase cAMP levels = bronchodilation

Question 25

What chemical is detected by newborn blood screen that points to cystic fibrosis?

Answer 25

immunoreactive trypsinogen

Question 26

What is the principal treatment for COPD?

Answer 26

Bronchodilators are the principal treatment for COPD, since corticosteroid treatment fails to relieve the inflammation associated with COPD. Inflammation has already had severe irreversible s/e

Question 27

How do COPD patients present?

Answer 27

-Gradually progressive dyspnea on exertion -Variable cough & sputum

Question 28

What’s the MOA of cromolyn?

Answer 28

mast cell degranulation inhibitor (specifically, inhibits chloride transport)

Question 29

What are the two commonly used anticholinergics used to treat COPD? Which is longer acting?

Answer 29

Ipratropium and tiotropium Tiotropium is long acting

Question 30

What are the only measures that can reduce mortality for COPD patients?

Answer 30

smoking cessation and supplemental oxygen

Question 31

What is ABPA (Allergic Bronchopulmonary Aspergillosis)?

Answer 31

-A hypersensitivity to Aspergillus fumigatus (fungus) -Occurs in asthma and cystic fibrosis almost exclusively

Question 32

What will you see on CT that points to bronchiectasis?

Answer 32

signet ring sign: bronchus is bigger than its accompanying artery.

Question 33

What’s the MOA of montelukast and zafirlukast?

Answer 33

block leukotriene receptor (CysLT1)

Question 34

What’s the MOA of Zileuton?

Answer 34

inhibits 5-lipoxygenase, the enzyme that converts arachidonic acid to leukotriene A4

Question 35

What’s the typical clinical presentation of an asthma patient?

Answer 35

-Intermittent dyspnea, cough, and wheezing. -Cough is sometimes the only sx -Nocturnal symptoms common = clue!!!

Question 36

In asthma cases, which med can you never use alone? what do you have to couple it with?

Answer 36

never use LABA alone, always couple with inhaled corticosteroids

Question 37

What are the important steroids used to treat asthma?

Answer 37

beclomethasone, fluticasone, and budesonide (first Aid)

Question 38

Which are more effective in controlling asthma: anticholinergics or adrenergic agonists?

Answer 38

adrenergic agonists are more effective than anticholinergics

Question 39

Which type of lymphocytes are particularly involved in the asthmatic immune response?

Answer 39

TH2 lymphocytes

Question 40

What is the most common mutation in the CFTR gene?

Answer 40

F508 deletion (3 base pair deletion that codes for phenylalanine at position 508)

Question 41

What’s the most common SABA (ideal for an acute asthma attack)?

Answer 41

albuterol

Question 42

Which are more effective in controlling COPD: anticholinergics or adrenergic agonists?

Answer 42

anticholinergics are more effective in COPD than are adrenergic agonists. They reduce vagal tone, which may lead to reversal of airway obstruction in narrowed airways of COPD patients.

Question 43

What’s the function of alpha-1 antitrypsin?

Answer 43

it’s a serine protease inhibitor that Inhibits the enzyme neutrophil elastase from damaging the lung tissue