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Clinical Manefestations > Ch. 15 > Flashcards

Ch. 15 Flashcards

1
Q

What are the pathological and structural changes associated with CF?

A

• Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree secondary to inadequate hydration of the periciliary fluid layer.
• Partial bronchial obstruction (mucous plugging)
• Hyperinflation of the alveoli
• Total bronchial obstruction (mucous plugging)
• Atelectasis
• Bronchiectasis

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2
Q

CF is the most common fatal inherited disorder in childhood?
True or False

A

True

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3
Q

What is CF?

A

An autosomal recessive gene disorder caused by mutations in a pair of genes located on a chromosome 7.

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4
Q

How many different mutations are in the gene in code for cystic fibrosis?

A

More than 1700

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5
Q

What is cystic fibrosis transmembrane conductance regulator CFTR?

A

It is more than one of the 1700 different mutations in the gene which codes for cystic fibrosis have been described.

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6
Q

What is a gating defect?

A

When the sodium channel does not open?

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7
Q

What is the conductance defect?

A

The sodium channel is open, but chloride does not move efficiently.

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8
Q

How is CF inherited?

A

CF is a recessive gene disorder. The child must inherit two copies of the defective CF gene, one from each parent (cystic fibrosis carriers) to have the disease.

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9
Q

What is the standard Mendelian pattern?

A

If both parents carry the CF gene the possibility of their children having CF (regardless of gender).

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10
Q

What are the two criteria that must be met to diagnose CF?

A
  1. Clinical symptoms consistent with CF in at least one organ system
  2. Clinical evidence of the CFTR dysfunction
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11
Q

What are the two test used to diagnose CF?

A
  1. sweat chloride test
  2. nasal potential difference
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12
Q

What is the value that the sweat chloride test has to be greater than?

A

Greater than 60mEq/L (on two occasions)

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13
Q

What is immunoreactive trypsin level (IRT)?

A

Is measured from the blood dots collected on all newborn infants on the Guthrie cards.

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14
Q

What are the clinical indicators for CF?

A
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15
Q

What is the sweat chloride test?

A

A reliable test for the identification of about 98% of patients with the CF. the number one test we use.

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16
Q

What is a pilocarpine?

A

A colorless, odorless, sweat producing chemical that is applied to the patient’s arm or leg (usually the forearm).

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17
Q

What is a genetic test?

A

A sample of the patient’s blood or cheek cells.

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18
Q

What are the sweat test interpretations for CF?

A
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19
Q

What is the nasal potential difference?

A

The impaired transport of sodium and chloride across the epithelial cells lining the airways of the patient with CF can be measured.

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20
Q

How can NPD be measured?

A

With a surface electrode over the nasal epithelial cells lining the inferior turbinate.

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21
Q

What does an increased NPD mean?

A

Strongly suggest CF

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22
Q

How are pregnant females offered screening for CF mutations?

A

Using a 32 to 85 mutation panel

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23
Q

What is the stool fecal fat test?

A

Measures the amount of fat in the infants stool, and the percentage of dietary fat that is not absorbed by the body

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24
Q

What is the normal value for a fecal fat test?

A

Greater than 300ug/g

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25
Q

What is the value of pancreatic insufficiency for infants for the fecal fat test?

A

Less than 50ug/g

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26
Q

What are the pathophysiologic mechanisms for CF?

A

• increased ventilatory rate
• stimulation of peripheral chemoreceptors (hypoxemia)
• decreasing lung compliance - increased ventilatory rate relationship
• anxiety
• increased temperature

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27
Q

What are the physical examinations for CF?

A

• Use of accessory muscles during inspiration and expiration
• pursed lip breathing
• barrel chest
• cyanosis
• digital clubbing
• peripheral edema and venous distention

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28
Q

What are some symptoms you can see with CF?

A

• Cough
• sputum production
• hemoptysis

29
Q

What are the chest assessment findings for CF?

A

• Decreased or increased tactile and vocal fremitus.
• Hyperresonant percussion note • diminished breath sounds
• crackles
• wheezing

30
Q

What is the percentage of spontaneous pneumothorax seen in adults with CF?

A

Greater than 20%

31
Q

What are the PFTs for moderate to severe CF (obstructive lung pathophysiology)?

A

Everything is decreased because they are not able to get air out

32
Q

What are the lung volume & capacity findings for moderate to severe CF (obstructive lung pathophysiology)?

A

Hyperinflation is most evident they are able to get air in but not out

33
Q

what will the ABG look like for mild to moderate stages of CF?

A

pH is increased everything else is decreased.

34
Q

What will the ABG look like for a severe stage of CF?

A
35
Q

What are the two dangerous ABG findings for CF?

A

• acute alveolar hyperventilation superimposed on chronic ventilatory failure
• acute ventilatory failure (acute hypoventilation) superimposed on chronic ventilatory failure

36
Q

What are the oxygen indices for moderate to severe stages of CF?

A
37
Q

What are the hemodynamic indices for moderate to severe stages of CF?

A
38
Q

What is the hematology for CF?

A

• increased hematocrit and hemoglobin (because of chronic hypoxemia)
• increased white blood cell count (due to infections)

39
Q

What are the electrolytes found in CF?

A

• Hypochloremia (chronic ventilatory failure)
• increased serum bicarbonate (chronic ventilatory failure)

40
Q

What is the sputum examination for CF?

A

Gram positive bacteria
- staphylococcus aureus
- Haemophilus influenzas
Gram negative bacteria
- pseudomonas aeruginosa (produces sweet smelling green sputum)

41
Q

What are the radiologic findings for CF?

A

• Translucent (dark) lung fields
• Depressed or flattened diaphragms
• Right ventricular enlargement (cor pulmonale)
• Areas of atelectasis and fibrosis (due to mucus plugging)
• Tram-tracks
• Bronchiectasis (often a secondary complication)
• Pneumothorax (spontaneous)
• Abscess formation (occasionally)

42
Q

What are tram tracks?

A

When the x-ray looks like railroad tracks due to destruction of mucus

43
Q

What is distal intestinal obstruction syndrome (DIOS)?

A

An intestinal obstruction (similar meconium ileus in neonates that occurs in older children and young adults with CF. The intestines are blocked and food does not digest properly causing malnutrition.

44
Q

What are the primary goals for CF?

A

• to prevent pulmonary infection
• reduce the amount of the bronchial secretions
• improve airflow
• provide adequate nutrition

45
Q

What do we use to reduce the amount of thick bronchial secretions in CF?

A

Dornase Alfa (dNASE)

46
Q

What does dNASE mean?

A

To break up

47
Q

What are the vitamins that will provide adequate nutrition for CF?

A

Vitamins A, D, E, K

48
Q

What are the respiratory care treatment protocols for CF?

A

• O2 therapy protocol
• airway clearance therapy protocol
• lung expansion therapy protocol
• aerosolized medication protocol
• mechanical ventilation protocol

49
Q

What does the oxygen therapy protocol do?

A
  1. Treats hypoxemia
  2. decreases WOB
  3. decreases myocardial work in patients with advanced pulmonary disease or acute exacerbation
50
Q

What does the airway clearance therapy protocol do?

A
  1. It clears excessive mucus production and accumulation
  2. Chest physiotherapy.
  3. Postural drainage
    High frequency chest wall oscillation (HFCWO) a.k.a. high frequency chest compression (HFCC)
51
Q

What does the lung expansion therapy protocol do?

A
  1. Helps offset the alveolar atelectasis associated with CF.
  2. Deep breathing and effective cough are key to reversing consolidation caused by mucous plugging
52
Q

What does the aerosolized medication protocol do?

A

Uses bronchodilators such as
- albuterol and Atrovent
- salmeterol or formoterol
- tiotropium
Uses mucolytic agents such as
- inhaled dNASE
- inhaled hypertonic saline
- Inhaled acetylcysteine

53
Q

What is the mechanical ventilation protocol do?

A

Uses noninvasive ventilation, such as BiPAP

54
Q

What are CFTR modulators for CF?

A

• correctors
• potentiators
• Ivacaftor

55
Q

What are correctors?

A

are drugs which help mutated CFTR reach the epithelial cell surface where the CFTR protein normally functions as a transmembrane regulator of chloride movement out of the cell and sodium transport into the cell.

56
Q

What are potentiators?

A

are drugs which help mutated CFTR function more effectively at the epithelial cell surface transporting chloride out of the cell and inhibiting the movement of sodium into the cell.

57
Q

What are the antibiotics used for CF?

A

Inhaled TOBI & ibuprofen

58
Q

What are the three organs that can have CF?

A

• pancreas
• lungs
• intestines

59
Q

What do CF and bronchiectasis have in common?

A

They both suffer from right sided heart failure

60
Q

What are the clinical manifestations for CF?

A

• Atelectasis
• bronchospasm
• excessive bronchial secretions

61
Q

When will you see a decrease tactile fremitus in CF patients?

A

Hyperinflation

62
Q

When will you see an increased, tactile fremitus in CF patients?

A

Secretions

63
Q

What shape should the diaphragm be in a normal person?

A

Dome shaped

64
Q

What shape will the diaphragm be in a CF patient?

A

Flat

65
Q

What is the number one cause of bronchiectisis?

A

CF

66
Q

When can a child only receive manual CPT?

A

When they are a small baby (1yr old and less).

67
Q

When can a child where a vest?

A

When they are 12 to 36 months (a toddler)

68
Q

And what PFTs for moderate to severe cystic fibrosis (obstructive, lung pathophysiology) are high due to hyperinflation?

A

RV, FRC, TLC

Clinical Manefestations (20 decks)

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