Cystic Fibrosis Flashcards

1
Q

True or false: Cystic fibrosis affects all races, but mostly affects caucasians

A

True

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2
Q

Which gender is affected more by cystic fibrosis?

A

Males, but not by very much

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3
Q

CF is an autosomal (dominant/recessive) disease

A

Recessive

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4
Q

What phenotype does a person with one mutated CFTR gene have?

A

Carrier of CF, but not affected with the condition themselves

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5
Q

What is the most common mutation in CF?

A

delta 508

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6
Q

What is the median age at death of patients with CF?

A

34.1 years old

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7
Q

Where are cystic fibrosis transmembrane conductance regulators (CFTRs) located?

A

Sweat glands

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8
Q

What are the functional and physical presentations of mutations in CFTRs?

A

Functional: inability to move chloride out to the cell surface
Physical: thick and sticky mucus

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9
Q

Where are CFTR proteins located?

A

. Lungs
. Pancreas
. Intestines
. Liver
. Sweat glands
. Reproductive

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10
Q

How is screening for CF done in newborns?

A

Blood sample from heel is analyzed

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11
Q

What is considered the gold standard test for CF screening?

A

Sweat chloride test

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12
Q

What components are required for diagnosis?

A

Positive sweat test and clinical symptoms

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13
Q

Clinical presentation of CF in pancreas

A

. Deficiency of pancreatic enzymes, insulin
. Malabsorption, malnutrition, CF diabetes

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14
Q

Which of the following is not a goal of therapy when treating CF?
a. Airway clearance
b. Prevent and treat sinusitis
c. Prevent and treat pulmonary exacerbations
d. Adequately digest fats, proteins and complex carbs

A

d. Adequately digest fats, proteins and complex carbs

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15
Q

What are the four step that should be implemented during airway clearance?

A

. Bronchodilator
. Mucolytic
. Enzyme mucolytic
. Inhaled antibiotic

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16
Q

The four steps of airway clearance should be performed in conjunction with ________ therapy

A

Percussion

17
Q

Which of the following is a mucolytic? An enzyme mucolytic?
a. Inhaled hypertonic saline
b. Inhaled sodium bicarbonate (2%)
c. Inhaled N-Acetylcysteines
d. Inhaled dornase alfa

A

Mucolytic: a. Inhaled hypertonic saline (HyperSal)
Enzyme mucolytic: d. Inhaled dornase alfa (Pulmozyme)

18
Q

Recommended age for use of pulmozyme?

A

Dornase alfa is recommended for patients 6 years old and up

19
Q

CF Foundation recommends inhaled _______ or ______ to age ≥ 6 years old with mild-to-severe lung disease with persistent __________ in sputum cultures

A

Tobramycin or aztreonam, Pseudomonas

20
Q

Which inhaled antibiotic is recommended for ≥ 6 years old with persistent P. aeruginosa infection?

A

Azithromycin