Cystic Fibrosis Flashcards

1
Q

is an inherited disease of the secretory glands

A

Cystic Fibrosis (CF)

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2
Q

The cystic Fibrosis disease is characterized by

A

thick mucus secretions, particularly in the pancreas and lungs, as well as electrolyte abnormalities in sweat gland secretions.

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3
Q

Cystic Fibrosis is inherited from an

A

autosomal recessive trait

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4
Q

______________ results in the inability to transport small molecules across cell membranes, leading to dehydration of epithelial cells

A

Abnormality of the long arm of chromosome 7

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5
Q

For early detection during pregnancy, ________ or _________ can be performed.

A

Chorionic Villi Sampling or Amniocentesis

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6
Q

Females affected by Cystic Fibrosis may have

A

thick cervical secretions that limit sperm motility

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7
Q

Affected males may be unable to reproduce secondary to persistent plugging and blocking of the vas deferens by

A

Tenacious seminal fluid

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8
Q

For cystic Fibrosis situation, patients who desire to become pregnant may have options like

A

*Alternative insemination
*In Vitro fertilization

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9
Q

the Acinar cells of the pancreas normally produce

A

*lipase
*trypsin
*amylase

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10
Q

Absence of pancreatic enzymes in the duodenum results in

A

inability to digest fat, protein, and some sugars.

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11
Q

Bowel movements become large, bulky, and greasy

A

Steatorrhea

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12
Q

An increase in the intestinal flora and fat results in

A

an extremely foul odor

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13
Q

increased bulk of feces often leads to

A

protuberant abdomen

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14
Q

Fat-soluble vitamins, particularly these vitamins cannot be absorbed in the absence of fat absorption resulting in vitamin deficiency

A

*Vitamin A
*Vitamin D
*Vitamin E

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15
Q

Meconium in newborn is normally

A

thick and tenacious

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16
Q

organisms most frequently cultured in CF situations

A

*Staphylococcus aureus
*Pseudomonas aeruginosa
*Haemophilus Influenzae

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17
Q

__________ may occur as a result of absorption of air from alveoli behind blocked bronchioles.

A

Atelectasis

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18
Q

The level of chloride to sodium in perspiration is increased

A

two to five time above normal

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19
Q

The level of chloride to sodium in perspiration is increased two to five time above normal resulting in

A

“salty” perspiration

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20
Q

Cystic Fibrosis may be suspected in a newborn who fails to regain normal birth weight within

A

7 to 10 days of birth

21
Q

may occur because of inadequate peripheral tissue perfusion

A

Clubbed Fingers

22
Q

Clubbed fingers may occur because of

A

inadequate peripheral tissue perfusion

23
Q

Respiratory Infections due to Cystic Fibrosis develop around

A

4 to 6 months of age

24
Q

wheezing and rhonchi are often heard on

A

chest auscultation

25
Q

detects abnormal salt concentrations

A

sweat testing

26
Q

a normal concentration of sodium chloride in sweat is

A

20 mEq/L

27
Q

more than _______ of sodium chloride if diagnostic of CF

A

more than 60 mEq/L

28
Q

may be used to detect pancreatic enzymes and reveal the extent of pancreatic involvement

A

Duodenal secretions

29
Q

the easiest pancreatic enzyme to assay

A

trypsin content

30
Q

may be tested to determine the extent of Atelectasis and emphysema

A

Pulmonary function

31
Q

is a collaborative process to reduce the involvement of the pancreas, lungs, and sweat glands.

A

Therapeutic Management

32
Q

Cystic fibrosis (CF) is a genetic disorder that primarily affects

A

lungs and digestive enzymes (pancreas)

33
Q

CFTR

A

Cystic Fibrosis Transmembrane Conductance Regulator

34
Q

Individuals who inherit only one faulty gene are called as

A

“carriers”

35
Q

most common in what decent?

A

Caucasians of Northern Europe Descent

36
Q

digital clubbing is a sign of

A

hypoxia (low oxygen levels)

37
Q

refers to the abnormal enlargement and rounding of the fingertips or toes

A

Digital Clubbing

38
Q

is a sinonasal manifestation of cystic fibrosis

A

Nasal Polyps

39
Q

Signs and Symptoms of Cystic Fibrosis:

A

*persistent cough
*salty tasting skin
*digital clubbing
*frequent lung infections
*wheezing, or shortness of breath
*nasal polyps
*poor growth or poor weight gain

40
Q

can be generated using an ultrasonic compressor and administered via a nebulizer mask.

A

nebulized mist

41
Q

can be included in the mist
to help thin and loosen secretions

A

A mucolytic

42
Q

children may have such thick bronchial secretions that they are unable to cough them up, even after they have been thinned by mist or aerosol therapy, they need

A

Chest Physiotherapy

43
Q

How to diagnose Cystic
Fibrosis

A

*sweat testing
*duodenal analysis
*stool analysis
*pulmonary testing

44
Q

is a special procedure used to manage pancreatic insufficiency in
individuals with CF

A

pancreatic enzyme replacement therapy
(PERT)

45
Q

PERT involves taking

A

oral pancreatic enzyme supplements
with meals and snacks

46
Q

elevated blood pressure in the lungs,

A

pulmonary hypertension.

47
Q

Bacteria line the airways in diseases that cause permanent widening of the
large airways

A

bronchiectasis

48
Q

Nursing Diagnosis for Cystic Fibrosis

A

*Impaired Gas Exchange related to thick respiratory secretions
* Ineffective Airway Clearance related to copious bronchial secretions as evidenced by nasal polyps
* Acute Pain related to bowel blockage
* Risk For Deficient Fluid Volume related to excessive fluid loss
* Imbalanced Nutrition related to malabsorption

49
Q

________ of the chest becomes enlarged in CF

A

The anteroposterior diameter of the chest