2.6 - Crr - Week 7

Front 1

what is RDS

Back 1

Respiratory Distress Syndrome, seen in premature infants with no surfactant

Front 2

RDS aka...

Back 2

Hyaline membrane disease (HMD)

Front 3

Predict RDS/HMD by testing ____ _______.

Back 3

amniotic fluid ("cascade testing"; florescence polarization study)

Front 4

RDS treatment

Back 4

corticosteroids (before birth), exogenous surfactant (aerosolized, sprayed into lungs)

Front 5

RDS complication: Repeated collapse of alveoli leads to trauma which ultimately leads to...

Back 5

fibrin-rich exudate leaking into alveolus causin hyaline membranes (and thick alveolar walls)

Front 6

Other complications of RDS (3)

Back 6

- blindness (Stevie Wonder; oxygen toxicity, retina problems)
- bronchopulmonary dysplasia (rare now; oxygen toxicity but ventilation is better now)
- risk of cerebral intravascular hemorrhage (very premature babies)

Front 7

adenomatoid malformation (describe)

Back 7

cystic hamartoma of the lung: lobe of the lung grows as a misshapen cyst.

Front 8

Most common mutation in cystic fibrosis (CF)

Back 8

delta-F-508

Front 9

gene effected in CF

Back 9

CFTR (cystic fibrosis transmembrane regulator)

Front 10

Most common lethal mutation in caucasians

Back 10

delta-F-508 (disease = CF)

Front 11

CF causes continual infection, dilating the bronchi, termed, "______."

Back 11

bronchiectasis

Front 12

transudate and exudate share this common characteristic by definition

Back 12

fluid in an inappropriate place

Front 13

transudate vs. exudate

Back 13

Transudate = LOW protein content
Exudate = HIGH protein content . . . "EXtra protein" (causes hyaline membranes allowing protein deposition)

Front 14

Kerley B lines indicate

Back 14

pulmonary edema

Front 15

Causes of pulmonary edema (2)

Back 15

- Left sided heart failure (increased pulmonary pressure)
- toxic injury leading to increased capillary permeability (risk of ARDS from fibrin leakage)

Front 16

4 steps of acid-base analysis

Back 16

1 - pH (high/low)
2 - Metabolic/Respiratory
3 - Anion Gap
4 - Appropriate and Effective Compensation?

Front 17

Alkalemia if pH is greater than

Back 17

7.44

Front 18

Acidemia if pH is less than

Back 18

7.36

Front 19

RESPIRATORY alkalemia if PaCO2 is less than

Back 19

36 mmHg (around there...this is according to Dr. Simpson)

Front 20

METABOLIC alkalemia if HCO3 is greater than

Back 20

27 meq/L (around there...this is according to Dr. Simpson)

Front 21

RESPIRATORY acidemia if PaCO2 is greater than

Back 21

40 mmHg (around there...this is according to Dr. Simpson)

Front 22

METABOLIC acidemia if HCO3 is less than

Back 22

24 meq/L (around there...this is according to Dr. Simpson)

Front 23

Formula for calculating the anion gap

Back 23

Anion Gap = Na - (Cl + HCO3)

i.e.

Anion Gap = Sodium - (Chloride + Bicarbonate)

Front 24

Normal anion gap value

Back 24

less than 12

Front 25

Mnemonic for causes of Anion Gap elevation

Back 25

KUSSMALE: ketoacidosis (diabetic and EtOH related), uremia, salicylates, starvation, methanol, aldehydes, lactate, ethylene glycol (antifreeze)

Front 26

If compensation is at work, how should PaCO2 and HCO3 values be shifted?

Back 26

They will be shifted in the SAME direction (both elevated/decreased)

Front 27

"Normal" PaCO2 value on ABG (according to Dr. Simpson)

Back 27

36 mmHg (around there...this is according to Dr. Simpson)

Front 28

"Normal" HCO3 value on ABG (according to Dr. Simpson)

Back 28

24 meq/L (around there...this is according to Dr. Simpson)

Front 29

METABOLIC acidosis: Equation to estimate change in PaCO2 value during compensation.

Back 29

delta-PaCO2 = 1.3(delta-HCO3)

Hint: Think the HIGHER number (1.3) goes with an abnormally low HCO3 value because we want it to be HIGHER.

Note: please remember that "delta" represents "change in..." (It looks like a triangle)

Front 30

METABOLIC alkalosis: Equation to estimate change in PaCO2 value during compensation

Back 30

delta-PaCO2 = 0.6(delta-HCO3)

Hint: Thing the LOWER number (0.6) goes with an abnormally high HCO3 value because we want it to be LOWER.

Note: please remember that "delta" represents "change in..." (It looks like a triangle)

Front 31

RESPIRATORY acidosis: Equation to estimate change in HCO3 value during compensation in an ACUTE setting.

Back 31

delta-HCO3 = .1(delta-PaCO2)

or

delta-HCO3 = .2(delta-PaCO2)

Hint: Same as alkalosis. "Small" numbers (.1, .2) go with "short" and "acute."

Front 32

RESPIRATORY acidosis: Equation to estimate change in HCO3 value during compensation in an CHRONIC setting.

Back 32

delta-HCO3 = .4(delta-PaCO2)

or

delta-HCO3 = .5(delta-PaCO2)

Hint: Same as alkalosis. "Big" numbers (.4, .5) go with "long" and "chronic."

Front 33

RESPIRATORY alklalosis: Equation to estimate change in HCO3 value during compensation in an ACUTE setting.

Back 33

delta-HCO3 = .1(delta-PaCO2)

or

delta-HCO3 = .2(delta-PaCO2)

Hint: Same as acidosis. "Small" numbers (.1, .2) go with "short" and "acute."

Front 34

RESPIRATORY alklalosis: Equation to estimate change in HCO3 value during compensation in an CHRONIC setting.

Back 34

delta-HCO3 = .3(delta-PaCO2)

or

delta-HCO3 = .4(delta-PaCO2)

Hint: Same as alkalosis. "Big" numbers (.3, .4) go with "long" and "chronic."

Front 35

If a patient is compensating as expected for an acidosis/alkalosis, but the pH is STILL off, what does this mean?

Back 35

There are at least 2 processes at work.

Front 36

3 possible causes of an acidosis with a normal anion gap ("hyperchloremic acidosis")

Back 36

- Kidney
- Bowel
- Resuscitation w/ normal saline

Front 37

Oxygen "carrying capacity" of blood

Back 37

Focuses on hemoglobin: 1.36mL/g multiplied by grams of Hb...

Front 38

Oxygen "content"

Back 38

((O2 bound to Hb)(% saturation)) + dissolved O2 in plasma

Front 39

Direction of shift in Hb-dissociation curve indicating a higher affinity

Back 39

Left shift of the Hb-dissociation curve

Front 40

Direction of teh shift in the Hb-dissociation cure indicating a lower affinity

Back 40

Right shift of the Hb-dissociation curve

Front 41

Four things which cause a right-shift of the Hb-dissociation curve

Back 41

- Increased [H+] (lower pH)
- Increased temperature
- Increased [DPG]
- Increased PCO2

Front 42

Most CO2 carried through the bloodstream via _______

Back 42

- bicarbonate (80-90%)
- carbamino Hb (7%)
- dissolved (4%)

Front 43

Define stagnant hypoxia

Back 43

normal PaCO2; low PvO2 (lower than normal venous oxygen). Cuased by slow peripheral blood flow (heart failure, shock, local obstruction)

Front 44

define hypoxic hypoxia

Back 44

low PaCO2 (low arterial oxygen). Caused by all the classic lung problems - interstitial disease, V/Q mismatch, COPD, high altitude, etc

Front 45

define histoxic hypoxia

Back 45

can't utilize oxygen. Cause by cyanide poisioning (blocks the electron transport chain).

Front 46

define anemic anoxia

Back 46

reduced functional hemoglobin. Caused by anemia, and CO poisioning.

Front 47

Describe the three pulmonary mechanoreceptors

Back 47

- stretch: in smooth muscle layers of airways. They terminate inspiration/expiration.
- irritant: between epithelial airway lining. Sensitive to smoke, cold air, dust, etc. (Plays a role in asthma bronchoconstriction?)
- J: "juxta-capillary." close to capillaries. can cause rapid shallow breathing and dyspnea.

Front 48

T or F: CF is autosomal recessive

Back 48

true

Front 49

on which chromosome is the CFTR gene?

Bonus points: long arm or short arm?

Back 49

7, long arm ("q" arm...b/c it's the next letter after "p" which stands for "petite" which is for the short arm.)

Front 50

Reproductive anomalies seen in CF

Back 50

- bilateral absence of vas deferens
- decreased ability of ova transit through fallopian tubes

Front 51

Kidney anomalies seen in CF

Back 51

- INCREASED ability to clear beta lactam antibiotics

Front 52

Sinus anomaly seen in CF

Back 52

severe chronic sinusitis

Front 53

Mechanistic pathway of pathology in CF

Back 53

1 - CF mutation
2 - thick mucus
3 - infection
4 - inflammation
5 - tissue destruction/bronchiectasis

Front 54

CF mutation can also lead to malabsorption/malnutrition. Which organ's fault is this and how to treat?

Back 54

Pancreas, first discovered that you can treat with ground-up pancreases from slaughtered livestock!

Front 55

Describe targeted CF treatment(s): Targeting the CF Gene and CFTR protein itself

Back 55

CF Gene:
• VX-770: drug that opens the Cl channels; however, it's so targeted at a specific genotype, it will only work with 2% of CF patients.

Front 56

Describe targeted CF treatment(s): Targeting the mucus itself and airway obstruction.

Back 56

Airway Obstruction (thick mucus):
• CPT/Airway Clearance: Pounding on chest and patient coughing. (CPT = chest physical therapy)
• rhDNase: discovered in the 60s that it's DNA that makes the thick secretions so goopy and sticky. Using animal rhDNase had miraculous effects until severe allergic rxns a couple weeks later. After recombinant human insulin started getting mnfctd, they tried it with human rhDNase, but not quite as miraculous effect.
• Hypertonic saline: based on the observation in Australia that CF patients who enjoyed surfing had better clinical outcomes.
• Albuterol: It helps everyone a bit.

Front 57

Describe targeted CF treatment options: Targeting infection.

Back 57

Infection:
• CPT/Airway Clearence: it also physically removes the bacteria, but keep in mind that there's naturally lots of bacteria in the lungs.
• Antibiotics: They help. Watch out for bigger and badder bacteria as we kill off the weaker ones.
• Vaccines: They help.

Front 58

Describe targeted CF treatment options: Targeting inflammation (the cause of lung destruction)

Back 58

Inflammation (key point of disease -- the cause of lung destruction):
• Steroids: helps with inflammation. Obviously. Mixed bag as well (just like ibuprofen) because you have to deal with all the side effects.
• Azithromycin: Also convenient that it's also anti-pseudomonas.
• Ibuprofen: MDs scared of the bleeding and other complications.

Front 59

Describe targeted CF treatment options: Treating actual tissue destruction

Back 59

Tissue Destruction:
• Lung Transplant: After all the other options, this is the very last one. $480,000 after transplant and care for first year. $80,000 for medications (per year?)

Front 60

Three steps to diagnose CF

Back 60

1 - Clinical syndrome
2 - Laboratory demonstration of CFTR defect (either abnormal sweat chloride test, or nasal potential difference)
3 - ID genetic mutation

Note with #2, there are other supportive - but not definitive - tests for CF diagnosis such as CXR, CT, PFTs, and stool studies.

Front 61

Common predisposing factor to pulmonary emboli

Back 61

deep vein thrombosis

Front 62

Common clinical presentation for PRIMARY (more rare) pulmonary hypertension

Back 62

dyspnea, 20-40 y/o female; caused by BMPR2 mutation

Front 63

describe Goodpasture's syndrome

Back 63

anti-glomerular basement membrane antibody; glomerulonephritis, and pulmonary hemorrhage.

Front 64

describe Wegener's Granulomatosis

Back 64

necrotizing granulomas and vasculitis; c-ANCA positive; renal and upper airway disease too