Congenital and Vascular Pulmonary Disease Flashcards

1
Q

Congenital Disorder refers to a…

A

disease or physical abnormality present from birth

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2
Q

Cystic Fibrosis (CF) is a disorder of ion transport of ________ in the exocrine glands of the liver, pancreas, intestines, reproduction and respiratory systems

A

Na & Cl

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3
Q

CFTR is a gene involved in production of…

A

sweat, mucus and digestive fluids

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4
Q

In CF, the CFTR gene is _________ leading to ______ secretions

A

nonfunctional

thick

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5
Q

CFTR works by moving ____ into the extracellular space to allow H2O to move out of the cell - ____ will thin mucus and make it easier for body to move mucus out

A

Cl

H2O

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6
Q

Know that CF is not exclusively a pathology of the _____!!!!

A

Lungs

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7
Q

CF is a ___________ disease

A

multisystem

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8
Q

Life expectancy of CF is what?

A

37.5 years

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9
Q

CF results in what issues?

A
  • Obstructive lung Disease - progressive loss of pulmonary functions
  • Digestive disorders
  • Chronic bacterial airway infections
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10
Q

Management of CF

A
  • Chest drainage
  • Bronchodilators & mucolytics
  • Percussion Therapy
  • Diets rich in proteins and calories
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11
Q

Parenchyma refers to the ______ in the lungs

A

alveoli

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12
Q

Pulmonary edema is when the _______ space fills with fluid that has leaked in from the ________

A

interstitial

vasculature

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13
Q

The volume of the pulmonary interstitial space is very ______ so it can accommodate only ____ amounts of fluid

A

limited

small

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14
Q

As the interstitial space fills with fluid, that fluid moves into the ______ spaces decreasing the space available for __________

A

alveolar

gas exchange

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15
Q

Pulmonary Edema patient’s feel like they are breathing through _____

A

water

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16
Q

Causes of Pulmonary Edema

A
  • fluid overload
  • decreased albumin
  • lymphatic obstruction
  • increased capillary permeability
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17
Q

Early stages of Pulmonary Edema start with what?

A

persistent cough, slight dyspnea, diaphoresis, and intolerance to exercise

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18
Q

As Pulmonary Edema progresses, symptoms change how?

A
  • cough becomes more productive with blood
  • dyspnea becomes more acute
  • RR increases
  • audible wheezing
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19
Q

The presence of Pulmonary Edema is a _________!

A

medical emergency

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20
Q

In supine, the heart presses on the _____, limiting _________

A

lungs

expandability

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21
Q

PT implications for Pumonary Edema: Treatment is aimed at…

A
  • Enhancing gas exchange, reducing fluid overload
  • Strengthening & slowing heart beat
  • Increased O2 along with diuretics, diet, and fluid restriction
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22
Q

Acute Respiratory Distress Syndrome (ARDS) is a condition that causes _____ to leak into your lungs, limiting movement of air into _______

A

fluid

alveoli

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23
Q

True or False, most people who get ARDS are already in the hospital with something else?

A

True, including sepsis, accidents, COVID or pulmonary toxic molecules

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24
Q

ARDS is characterized by what?

A

widespread inflammation of the lungs

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25
Q

ARDS leads to threatening ________!

A

hypoxemia

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26
Q

True or False, ARDS has a low mortality rate?

A

False! the mortality rate is high at 20-50%

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27
Q

Atelectasis is the _______ of normally expanded lung tissue at any structural level

A

collapse

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28
Q

Primary cause of obstructive-absorptive is obstruction of the _______ serving the affected area

A

bronchus

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29
Q

In Atelectasis, air in the alveoli is not ______, causing the alveoli to _______

A

replaced

collapse

30
Q

Causes of Atelectasis include:

A
  • hypoventilation
  • failure to breathe deeply postop
  • oversedation, coma, immobility
  • loss of surfactant
31
Q

PT implications for Atelectasis

A
  • Frequent position changes
  • Deep breathing
  • Coughing with pillow chest splint
  • Ambulating
32
Q

Pneumothorax is the _______ collection of air in the ______ space

A

abnormal

pleural

33
Q

Is pneumothorax bilateral or unilateral?

A

unilateral

34
Q

How is air removed in Pneumothorax?

A

syringe or chest tube

35
Q

Pneumothorax can only develop if air is allowed to enter the pleural space, through damage to the ________ or damage to the _____ itself

A

chest wall

lung

36
Q

Pathophysiology of Pneumothorax

A

Air is accumulated in the pleural space–> compressing the lung–> limiting amount of air inhaled

37
Q

What are the two types of Lung Cancer

A

Small cell lung caner (SCLC) and Non-small cell lung cancer (NSCLC)

38
Q

In SCLC, cells become so dense that there is almost no _______ present, so the cells are compressed into an ovoid _____

A

cytoplasm

mass

39
Q

SCLC tends to be located _______ near the _____ of the lung

A

centrally

Hilum

40
Q

________ and distant ________ are usually present at the time of SCLC diagnosis

A

Lymphatic

metastasis

41
Q

SCLC occurs most frequently in ______

A

smokers

42
Q

NSCLC involves _______ and ______

A

lymph and blood vessels

43
Q

NSCLC metastases in the….

A

brain, bone and liver

44
Q

NSCLC carcinomas of what organs are likely to metastasize to the lung?

A

kidneys, breast, pancreas, colon, and uterus

45
Q

3 main subtypes of NSCLC

A
  • squamous cell carcinoma
  • Adenocarcinoma
  • Large cell
46
Q

Squamous cell carcinoma is ______%; cells lining the __________

A

25-30%

passageways

47
Q

Adenocarcinoma is ___%; cancer of the cells that secrete substances like _______

A

40%

mucus

48
Q

Large cell appears anywhere in the ______ and grows _____

A

lung

quickly

49
Q

What is the most common lung cancer?

A

NSCLC

50
Q

In NSCLC, tumors in the ____ of the lungs can invade the _______, destroying ribs 1&2, causing _____ involvement

A

apex
brachial plexus
phrenic

51
Q

Lung Cancer Diagnosis: most are detected on routine _________ taken for ______ issues

A

chest X-ray

unrelated

52
Q

Clinical manifestations of Lung Cancer

A
  • Cough, sputum production and dyspnea
  • Anorexia, fatigue, weakness, weight loss
  • Recurring bronchitis or pneumonia
  • Difficulty swallowing
  • Cardiac and Esophageal compression
53
Q

Treatments for Lung Cancer

A
  • Surgical but not usually for SCLC b/c of location

- Radiation & Chemo

54
Q

Pulmonary HTN is defined as:

A

high BP in the pulmonary arteries

55
Q

What is normal pulmonary BP and what is elevated?

A
  • normal= 15-18 mmHG

- elevated= 5-10 ABOVE normal

56
Q

Pulmonary HTN is characterized by diffuse __________ of the pulmonary arteries caused by _________ of smooth muscle in the vessel walls and formation of _______ lesions in and around the vessels

A

narrowing
hypertrophy
fibrous

57
Q

Pathophysiology of pulmonary HTN

A

vasoconstriction

58
Q

Causes of Pulmonary HTN

A
  • CHF
  • Blood clots in lungs
  • HIV
  • Cocaine or meth use
  • Liver disease
  • CT disorders
  • Lung diseases
  • Congenital heart diseases
59
Q

Treatment of pulmonary HTN is predicated on _____

A

cause

60
Q

Prognosis of Pulmonary HTN

A

poor w/out heart-lung transplant

-2-3 years

61
Q

A Pulmonary embolism is when a clot in the ______ system dislodges and causes a blockage in the __________ circulation

A

venous

pulmonary

62
Q

PE causes ________ influences on health

A

long-term

63
Q

Does a PE impact the left or right side of the heart?

A

Right

64
Q

Pulmonary embolism downstream effect:

A

infarction of lung tissue–> acute pulmonary HTN–> increased demand on R side heart–> some degree of R heart dysfunction

65
Q

PE risk factors

A
  • prolonged immobility
  • hypercoagulability
  • damage to the walls of veins
66
Q

Signs and Symptoms of PE

A
  • apprehension
  • diaphoresis
  • chest pain/tightness
  • cough
  • tachypnea
  • tachycardia
  • cyanosis
  • hypotension
67
Q

Lung transplantation types:

A

-Single lung transplant: posterolateral thoracotomy
-Double lung transplant:
bilateral anterior thoracotomies ; “clam shell”, imagine lifting the hood of a car
-Heart-lung transplant: Midline sternotomy

68
Q

There needs to be a match in ____ between donor and recipient for a lung transplant

A

size

69
Q

Anti-rejection drugs ________ the immune system

A

suppress

70
Q

Glucocorticoids AEs:

A
  • proximal muscle weakness
  • Hyperglycemia/diabetes
  • OP
  • Skin thinning
  • Impaired wound healing
  • Water retention
71
Q

PT implications for Lung transplant recipients:

A
  • pts are deconditioned
  • wound management
  • lots of lines/wires
  • slow improvement
  • exercise/physical function activities