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M2M Unit IV > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

How many disease-causing mutations have been identified in the CF gene?

A

1500

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1
Q

What are some respiratory problems associated with CF?

A

chronic sinus and respiratory infections, Bronchiectasis

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2
Q

What is the problem with the pancreas in CF?

A

failure of the pancreas to produce sufficient digestive enzymes to allow breakdown and absorption of fats and protein; also can have CF related diabetes

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2
Q

What is Ivacaftor?

A

a CFTR potentiator given to pts with at least one G551D or gating mutation

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4
Q

What is the median life span of pts with CF?

A

37

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4
Q

What is Lumacaftor?

A

a CFTR corrector for pts with 2 copies of the F508del mutation

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5
Q

What is tobramycin?

A

an inhaled antibiotic

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5
Q

What happens to the periciliary layer in CF pts?

A

the cilia cannot beat b/c they are so bogged down with thick mucus

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6
Q

CF should also be considered in infants and children who present with severe _____ and hypochloremic metabolic alkalosis.

A

dehydration

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6
Q

What are the airway management treatments for CF?

A

Daily percussive therapy, Recombinant human DNAse, Inhaled hypertonic saline, azithromycin, and Bronchodilators

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8
Q

Why are most male CF pts infertile?

A

the vas deferens doesn’t develop

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9
Q

What is steatorrhea?

A

excess fat in the feces

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10
Q

Infants with undiagnosed CF may also present with hypoproteinemia with or without edema, _____, and deficiency of _____.

A

anemia; the fat-soluble vitamins

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11
Q

An acute change in respiratory signs and symptoms from the subject’s baseline is called a _________.

A

pulmonary exacerbation

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12
Q

The most common CF mutation is the _______.

A

F508del

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12
Q

What is bronchiectasis?

A

chronic enlargement of airways in the lungs (bad)

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12
Q

CF should also be considered in infants and children who present with severe dehydration and _______.

A

hypochloremic metabolic alkalosis

12
Q

The CFTR functions as an ______ and controls the movement of ____ and _____ into and out of cells.

A

ion channel; salt; water

13
Q

Name 2 CF physical signs.

A

clubbing of the fingernails; nasal polyps

15
Q

What is the result of a defective or deficient CFTR?

A

abnormally thick secretions in various organ systems and critically altering host defense in the lung

17
Q

_______ occurs in more than 85% of persons with CF.

A

Pancreatic insufficiency

18
Q

What is unique about the stools in CF pts?

A

they’re greasy, bulky, and malodorous

19
Q

Pancreatic insufficiency occurs in more than ____ of persons with CF.

A

85%

21
Q

How is CF inherited?

A

autosomal recessive

22
Q

How is inflammation treated in CF?

A

high dose ibuprofen

24
Q

CF is caused by a ________ gene.

A

defect in an ATP-binding cassette transporter

26
Q

What is the false negative rate for CF?

A

1-5%

26
Q

Approximately 15% of newborns with CF present at birth with ______.

A

meconium ileus

27
Q

Why do untreated CF pts present with failure to thrive?

A

exocrine pancreas insufficiency

28
Q

What is a pulmonary exacerbation?

A

An acute change in respiratory signs and symptoms from the subject’s baseline

29
Q

What is an issue with the liver in CF?

A

focal sclerosis

31
Q

What eventually becomes the predominant pathogen in lung infections in CF patients?

A

Pseudomonas aeruginosa

32
Q

What are the dietary treatments for CF?

A

enzyme supplementation, high calorie, high protein, high fat diet, fat soluble vitamin supplements, and salt supplements

33
Q

Infants with undiagnosed CF may also present with ______ with or without ____, anemia, and deficiency of the fat-soluble vitamins.

A

hypoproteinemia; edema

34
Q

The _____ functions as an ion channel and controls the movement of salt and water into and out of cells.

A

CFTR

35
Q

What is meconium ileus?

A

severe intestinal obstruction resulting from inspissation of tenacious meconium in the terminal ileum

37
Q

What is the diagnostic sweat chloride level in CF?

A

> 60 mmol/L

38
Q

What is the incidence rate of CF?

A

1:3000 Caucasians; 1:9000 Hispanics

39
Q

Name the fat soluble vitamins.

A

A, D, E, and K

40
Q

Where are the CF genes located?

A

chromosome 7

41
Q

What is aztreonam?

A

an inhaled antibiotic

M2M Unit IV (11 decks)

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