RESPIRATORY DISORDERS Flashcards

1
Q

what is cystic fibrosis?

A

Cystic fibrosis is an inherited condition in which the lungs and digestive system can become clogged with thick, sticky mucus.

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2
Q

explain cystic fibrosis in a molecular level

A

misfolded> can’t migrate from the ER to the Cell membrane> Lack of CFTR protein

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3
Q

Explain in order the organs affected in cystic fibrosis?

A

NewBorn>> colon

Early childhood>> pancreas

Adults>> lungs

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4
Q

what is another name for cystic fibrosis?

A

“fibrocystic disease of the pancrea”

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5
Q

explain in detail what happens to the newborns in cystic fibrosis?

A
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6
Q

explain in detail what happens to the pancreas in cystic fibrosis?

A

backed up pancreatic enzymes can digest the pancreatic duct.

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7
Q

explain in detail what happens to the lungs in cystic fibrosis?

A
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8
Q

what test do we perform to confirm the diagnosis of cystic fibrosis and why?

A

Sweat test> abnormally salty sweet

the anscence of the CFTR on the apical membrane of the epithelial cells lining the duct of sweat glands, results in poor re-absorption of the chloride ions. Cl-

therefore, the sweat is rich in Cl- & Na+ ions

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9
Q

what other issues in the body may be caused by cystic fibrosis? (3)

A
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10
Q

why r men w/ cystic fibrosis infertile?

A

due to abscence of the VAS DEFERNS

Vas Deferens: the duct which conveys sperm from the testicle to the urethra.

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11
Q

what lung problems can occur from cystic fibrosis?

A
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12
Q

due to cystic fibrosis, what can the chronic bacterial infection and inflammation lead to?

A

occasionally, if the inflammation erodes into a BV > hemoptysis (coughing up blood)

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13
Q

Diagnosis of CF?

A
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14
Q

difference in CFTR roles in sweat glands vs lungs and pancreas?

A

unlike in lungs an pancreas where chloride can get out (into the mucous) , when CFTR is not working in the sweat glands, Cl- can’t come in! or be re-absorbed >> making sweat tastes salt.

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15
Q

Treatment of CF?

A

chest physiotherapy: pats on chest to loosen up mucous

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16
Q

Personalized treatments of CF?

A
17
Q

what kind of disorder is CF? (genetic level) epidemiology?

A
18
Q

Identify 4 common cystic fibrosis complications and describe how they are treated

A

- Respiratory Infections – aggressive therapy with physio and prophylactic antibiotics

- Low body weight – pancreatic enzyme replacement therapy, high calorie intake and extra supplements

- Distal Intestinal Obstruction Syndrome (DIOS)

  • CF Related Diabetes
19
Q

Describe the management of cystic fibrosis

A
  • Avoid smoking
  • Avoid other CF patients
  • Avoid jacuzzis (pseudomonas)
  • Annual influenza immunisation
  • Sodium chloride tablets in hot weather / vigorous exercise