Cystic Fibrosis Flashcards

1
Q

What Is an inherited disorder in which a defective gene affects the cells that produce mucus, sweat, and secretions used in digestion?

A

Cystic Fibrosis

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2
Q

What organ systems does CF primarily effect?

A

GI and respiratory

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3
Q

What is the most common complication of cystic fibrosis?

A

Acute and chronic respiratory infection

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4
Q

What is the first symptom of cystic fibrosis in newborns?

A

Meconium ileus

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5
Q

What is the gold standard for diagnosing cystic fibrosis?

A

sweat test

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6
Q

What does the sweat test measure?

A

chloride

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7
Q

What is a mucolytic enzyme that can help break apart extracellular DNA left behind in the mucus by neutrophils and other cells involved in inflammation?

A

Dornase alfa

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8
Q

What helps open the airways and prevent bronchospasm?

A

Albuterol

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9
Q

What is the most common bacteria to infect the lungs with patients with Cystic Fibrosis early in life?

A

S. aureus

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10
Q

What is the most common bacteria to infect the lungs with patients with Cystic Fibrosis later in life?

A

P. aeruginosa

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11
Q

What 2 drugs are given with chronic P. aeruginosa?

A

tobramycin or aztreonam

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12
Q

What is the Recommended Order of Chronic Inhaled Therapies for Cystic Fibrosis?

A

Bronchodilator to Hypertonic saline to Dornase alfa to Airway clearance techniques to Inhaled antibiotics

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13
Q

What Is a monoclonal antibody sometimes used to help prevent RSV in newborns during their first two years?

A

Palivizumab

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14
Q

What is the ADR of pancreatic enzymes for long periods of time?

A

tissue scarring in the gastrointestinal tract and narrowing of the colon

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15
Q

What age group typically gets Cystic fibrosis-related diabetes?

A

18-21 year olds

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16
Q

What is used as the First-line therapy for most patients with cystic fibrosis-related diabetes?

A

Insulin

17
Q

What is first-line therapy for Depression and Anxiety?

A

SSRIs

18
Q

What medication works on the cystic fibrosis transmembrane conductance regulator protein that binds to and opens the defective ion channel?

A

Ivacaftor

19
Q

What type of cancer are CF patients predisposed to?

A

Colorectal cancer

20
Q

What is the most commonly used bronchodilator?

A

Albuterol

21
Q

What drug allows endogenous proteolytic enzymes to break down the proteins, thus decreasing viscoelasticity and surface tension of purulent sputum?

A

Dornase Alfa