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Biology 253 > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

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1
Q

What is Cystic Fibrosis (CF)?

A

Defective chloride transport channel on CM

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2
Q

CM is composed of ______ and ______ making up the _______ layer. Heads of CM are _______ and tails are _______.

A

lipids and proteins

mosaic

hydrophobic

hydrophyllic

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3
Q

What are the 2 types of protein found on CM? Are they on outside or inside of CM?

A
  1. Extrinsic proteins–> outside of CM

2. Intrinsic proteins –>inner part of CM

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4
Q

Extrinsic and intrinsic proteins make up the _____ which move ___ and _______ in and out of cells.

A

channels

ion

substances

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5
Q

Etiology of CF (3)

A
  1. CFTR gene mutation on Chr. 7
  2. autosomal recessive
  3. increase rate in caucasian population
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6
Q

What does the CFTR gene stand for and function?

A

CFTR gene = cystic fibrosis transmembrane regulated genes

Fxn: Code for protein that regulate the passage of ions across the membrane

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7
Q

How does CFTR gene mutation result in CF?

A

transmembrane exchange is altered specifically to the exchange of Cl- ions —> causing defective Cl- ion channels

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8
Q

What does autosomal recessive mean?

A

req two defective alleles

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9
Q

If Cl channels are defective, there are secretions of _____ ions not ____ ions. This affects ______ glands and is not restricted to the _________ system. The problem is _____ sepcific

A

sodium

Cl-

exocrine

respiratory

site

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10
Q

Patho of CF (3)

A
  1. gene code Cl- protein channel on CM
  2. mutations –> channel permeability altered
  3. abn exocrine sec (tissue specific)
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11
Q

Patho: Gene code for Cl- protein channels are mutated.

A

protein that is responsible for transporting Cl- ions are defective

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12
Q

Patho: How does the mutation affect the protein channels? What happens to the transport of Cl- ion?

A

Cl- transport channels are impaired –> abnormal secretion of Cl- (xs, minimal, or no transport of Cl- ions)

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13
Q

T or F: CF can affect exocrine and endocrine galnds

A

WRONGGGG

affects EXOCRINE gland ONLY

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14
Q

Patho: What does it mean by abn exocrine secretion is tissue specific?

A

What happens in one area may not occur in another area

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15
Q

What organ system does it affect (3)?

A
  1. GIT
  2. resp system

3, reproductive system

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16
Q

Fig. 29. 14: What is needed for mucus to from? (3)

What is the consistency with these in place?

A
  1. chloride
  2. sodium
  3. H2O

normal consistency: not too thick and not too sticky

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17
Q

Fig. 29. 14: In the respiratory tract, what happens if the Cl- ions are defective? Na and H2O?

A

no chloride ions secretion and accum in the cytoplasm –> increase in conc.–>Na and H2O secrete as normal BUT reabsorb into the cytoplasm–> leaves a thick, sticky, and viscous mucus

18
Q

Fig. 29. 14: thick and sticky mucus overwhelms the _________ ________ and _____ is unable to clear mucus

A

mucociliary blanket

cilia

19
Q

Fig. 29. 14: abn mucus composition creates a ______________ that protects _________ _________ such as ________ resulting in infection and ______.

A

microenvironment

foreign microbes

bacteria

inflm –> release proteases which cause tissue destruction

20
Q

Fig. 29. 14: What can result with decrease mucus clearance and abn mucus composition?

A
  1. chronic a/w obstruction

2. bacterial infection

21
Q

Complications of CF (3)

A
  1. chronic bronchitis
  2. bronchiectasis
  3. respiratory failure
22
Q

Respiratory Tract in CF: How does thick mucus lead to impaired breathing? What else can occur? What causes death?

A
  1. thick mucus –> compromised ciliary function–> a/w obstruction–> impaired ventilation—> no GE —> impaired breathing
  2. bacterial infection
  3. death from severe pulm complication
23
Q

Respiratory Tract in CF: Why is breathing impaired?

A

no GE—> hypoxemia—> systemic hypoxia and hypercapnia –> dyspnea

24
Q

Respiratory Tract in CF: abn composition of mucus create an ideal ________ for _______ to establish and cause _________ b/c inhaled microbe cannot be _________

A

medium

bacteria

pneumonia

expelled

25
Q

Respiratory Tract in CF: _____ from severe pulm complications.

A

death

26
Q

Diagnosis for CF (4)

A
  1. sweat test
  2. newborn screen (trypsinogen)
  3. look for resp and GI mnfst
  4. sibling hx
27
Q

How is the sweat test used?

A

Sweat test–> analyze the perspiration of the sweat —>measures the conc. of sodium chloride concentration

28
Q

In CF, conc. of NaCl is ___-___ time higher b/c most NaCl remain in the _____ b/c there are _____ to ___ withdrawal of Cl- ions moving out of the _____ ___

A

2-5x higher

duct

little to no

secretory cell

29
Q

In a normal individual: Na and Cl move from _______ into the _______ cells, as secretion moves down, certain quantity of Cl- ions is _______ out of secretory cells and Na _____.

A

circulation

secretory cells

removed

follows

30
Q

In a normal individual: most NaCl are ______ from secretion and some _____ in perspiration

A

removed

remain

31
Q

What is the newborn screen? Trypsinogen increase or decrease?

A

It is a blood test screening in newborns to detect increase levels of trypsinogen d/t secretory obstruction in pancreas

32
Q

Trypsinogen is a ______ for ____ which break down ______

A

precursor

trypsin

proteins

33
Q

GI Manifestations (3)

A
  1. diarrhea
  2. abdominal pain
  3. abn pancreatic secretion
34
Q

Screening test are done esp if newborns’s ________ have _____ _______. Why?

A

siblings

CF

strong possibility that newborn have it too

35
Q

Treatment of CF (8)

A
  1. no cure
  2. manage prog
  3. antibiotics and gamma gobulins for infection
  4. diet
  5. enzyme supplement
  6. mucolytic and anti-inflm
  7. chest physio
  8. DNAase
36
Q

There is no cure b/c it is a _____ prob

A

genetic

37
Q

__________ management

A

symptomatic

38
Q

What are gamma gobulins? How does it tx infection?

A

antibodies

use to prophylactically tx infection

39
Q

What is supplemented in diet? What is limited and increased in diet?

A

supplement NaCl in diet

limit fats

ensure high protein and calorie diet

40
Q

Why supplement enzymes?

A

d/t deficiency of pancreatic enzymes

41
Q

Why is chest physiotherapy used to tx CF?

A

to help dislodge and expectorate mucus

42
Q

Explain DNAase

A

an enzyme that breaks up the DNA strands present in mucus within the a/w making mucus more mobile to expectorate

Biology 253 (39 decks)

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