Final: Ch 23 CF, Restrictive Lung Disease, Pulmonary Embolism, PA HTN, ARDS, RF

Question 1

genetics of cystic fibrosis

Answer 1

autosomal recessive mutation

mutated CF transmembrane regulator channel (Cl-)

Question 2

impacts of CF on sweat glands and airways

Answer 2

sweat glands: CFTR doesn’t reabsorb secreted Cl-, so very salty sweat

airways: CFTR doesn’t allow Cl- to enter airways, causing lower water content of sputum

Question 3

respiratory and pancreatic symptoms of CF

Answer 3

respiratory: viscous sputum, chronic bronchitis, respiratory infections
pancreatic: may include exocrine pancreatic insufficiency

Question 4

diagnosis of CF

Answer 4

sweat test: conductivity and chem analysis

genetic test for CFTR mutation

Question 5

treatment of CF

Answer 5

postural drainage to clear secretions

antibiotics for infection

deoxytibonuclease breaks down DNA in sputum

oral replacement of pancreatic enzymes

Question 6

interstitial (restrictive) lung disease causes

Answer 6

inflammation and fibrotic changes to lung tissue

environmental, drugs, immune

alveolar macrophages secrete TGF and fibroblast GF

fibrosis results

Question 7

symptoms of interstitial (restrictive) lung disease

Answer 7

lower lung volumes and diffusion capacity

dyspnea, tachypnea, cyanosis

low FVC and TLC

normal or even high FEV1/FVC

Question 8

diagnosis and treatment of interstitial (restrictive) lung disease

Answer 8

Dx: CXR, biopsy

Rx: steroids for inflammation

Question 9

idiopathic pulmonary fibrosis

Answer 9

unknown cause, smoking risk factor

CXR: patchy infiltrates

survival 3-5 yrs

Question 10

sarcoidosis

Answer 10

systemic disorder

granulomas form in lung, nodes, or other organs

genetic or environmental

Question 11

symptoms of sarcoidosis

Answer 11

SOB

non-productive cough

chest pain

weight loss

fatigue

Question 12

Dx/Rx of sarcoidosis

Answer 12

Dx: H&P, CXR

Rx: remove cause, steroids

Question 13

pulmonary embolism

Answer 13

any blood-borne substance that lodges in the PA

ex. fat, amniotic fluid, air

Question 14

most common cause of pulmonary embolism

Answer 14

emboli from DVT of legs

Question 15

risk factors for DVT

Answer 15

venous stasis - immobilization

trauma

smoking

BCP

Question 16

symptoms of pulmonary embolism

Answer 16

chest pain

dyspnea

bloody sputum

tachycardia, tachypnea

can cause cardiovascular collapse

Question 17

Dx of pulmonary embolism

Answer 17

lung scans, CT, angiography

high D-diamer (fibrin degeneration product) indicates clotting

Question 18

Rx of pulmonary embolism

Answer 18

prevent DVT (anticoagulant therapy)

vena cava plication: insertion of filter in vena cava to catch clots

intermittent pneumatic compression boot

Question 19

normal pulmonary artery pressure is, and what causes elevations

Answer 19

28/8 (15 mean)

high resistance, blood flow, LAP

Question 20

2 types of PA HTN

Answer 20

primary

secondary

Question 21

primary PA HTN

Answer 21

rare, but progressive and deadly

triggers: HIV, autoimmune, hypoxia

familial is autosomal dominant mutation of TGF-beta receptor (SMC proliferation)

Question 22

secondary PA HTN

Answer 22

secondary to another disease

high pulmonary venous pressure (mitral stenosis, Lt HF)

high pulmonary blood flow (ASD, VSD, PDA)

high resistance (PE, hypoxic pulmonary vasoconstriction)

Question 23

symptoms of 2ndary PA HTN

Answer 23

dyspnea

peripheral edema

fatigue

Rt HF

Question 24

Dx/Rx of 2ndary PA HTN

Answer 24

Dx: CXR, echo, Rt heart cath

Rx: vasodilators

Question 25

Dx/Rx of primary PA HTN

Answer 25

Dx: pulmonary HTN without another cause

Rx: O2, Ca channel blockers, prostacyclin

Question 26

Cor Pulmonale

Answer 26

Rt HF from lung disease

PA HTN –> RV hypertrophy –> failure

Rx: treat lung disease, give O2

Question 27

ARDS

Answer 27

highly fatal

common pathway from a range of lung problems

Question 28

pathologic changes of ARDS

Answer 28

alveolar-capillary memb. disrupted

proteins, fluid, and WBC enter interstitium & alveoli to cause hypoxia

surfactant inactivated (hypoxia, alveolar collapse)

Question 29

Rx: of ARDS

Answer 29

supportive

give O2

use PEEP

Question 30

respiratory failure

Answer 30

failure of lungs to deliver O2, or to remove CO2 from blood

Question 31

causes of respiratory failure

Answer 31

impaired:

ventilation

diffusion

V/Q matcing

Question 32

hypoxemic respiratory failure

Answer 32

V/Q mismatch seen in COPD

severe hypoxia when low V/Q areas exist

impaired diffusion: thickening of respiratory memb.

O2 therapy

Question 33

hypercapnia/hypoxemic respiratory failure

Answer 33

hypoventilation decreases minute alveolar ventilation

central (drug OD, head injury), peripheral (Gullian-Barre), mucsular distrophy

produces hypercapnea but not hypoxia until severe

Question 34

symptoms of respiratory failure

Answer 34

hypoxia: cyanosis, seizures
polycythemia: when chronic hypoxia
hypercapnea: air hunger and headache

Question 35

Dx of respiratory failure

Answer 35

ABG

pulse oximeter

PA catheter for MV O2

Question 36

treatment of respiratory failure

Answer 36

O2 therapy

positive pressure mechanical ventilation

intubation of trachea

mask methods?

Question 37

CXR of pleural effusion vs pneumothorax

Answer 37

PE: white out on affected side

Pneumothorax: blackout on affected side

Question 38

Why does incentive spirometer work for atelectasis

Answer 38

Produces negative intrapleural pressure to pull open collapsed alveoli

Question 39

PA HTN produces rt or lt HF?

Answer 39

Rt HF

Question 40

Distinguish obstructive from restrictive lung disease

Answer 40

Obstructive has a low FEV1/FVC

restrictive has a normal to high FEV1/FVC