Cystic Fibrosis

Question 1

is an inherited disease of the secretory glands

Answer 1

Cystic Fibrosis (CF)

Question 2

The cystic Fibrosis disease is characterized by

Answer 2

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

Question 3

Cystic Fibrosis is inherited from an

Answer 3

autosomal recessive trait

Question 4

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

Answer 4

Abnormality of the long arm of chromosome 7

Question 5

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

Answer 5

Chorionic Villi Sampling or Amniocentesis

Question 6

Females affected by Cystic Fibrosis may have

Answer 6

thick cervical secretions that limit sperm motility

Question 7

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

Answer 7

Tenacious seminal fluid

Question 8

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

Answer 8

*Alternative insemination
*In Vitro fertilization

Question 9

the Acinar cells of the pancreas normally produce

Answer 9

*lipase
*trypsin
*amylase

Question 10

Absence of pancreatic enzymes in the duodenum results in

Answer 10

inability to digest fat, protein, and some sugars.

Question 11

Bowel movements become large, bulky, and greasy

Answer 11

Steatorrhea

Question 12

An increase in the intestinal flora and fat results in

Answer 12

an extremely foul odor

Question 13

increased bulk of feces often leads to

Answer 13

protuberant abdomen

Question 14

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

Answer 14

*Vitamin A
*Vitamin D
*Vitamin E

Question 15

Meconium in newborn is normally

Answer 15

thick and tenacious

Question 16

organisms most frequently cultured in CF situations

Answer 16

*Staphylococcus aureus
*Pseudomonas aeruginosa
*Haemophilus Influenzae

Question 17

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

Answer 17

Atelectasis

Question 18

The level of chloride to sodium in perspiration is increased

Answer 18

two to five time above normal

Question 19

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

Answer 19

“salty” perspiration

Question 20

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

Answer 20

7 to 10 days of birth

Question 21

may occur because of inadequate peripheral tissue perfusion

Answer 21

Clubbed Fingers

Question 22

Clubbed fingers may occur because of

Answer 22

inadequate peripheral tissue perfusion

Question 23

Respiratory Infections due to Cystic Fibrosis develop around

Answer 23

4 to 6 months of age

Question 24

wheezing and rhonchi are often heard on

Answer 24

chest auscultation

Question 25

detects abnormal salt concentrations

Answer 25

sweat testing

Question 26

a normal concentration of sodium chloride in sweat is

Answer 26

20 mEq/L

Question 27

more than _______ of sodium chloride if diagnostic of CF

Answer 27

more than 60 mEq/L

Question 28

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

Answer 28

Duodenal secretions

Question 29

the easiest pancreatic enzyme to assay

Answer 29

trypsin content

Question 30

may be tested to determine the extent of Atelectasis and emphysema

Answer 30

Pulmonary function

Question 31

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

Answer 31

Therapeutic Management

Question 32

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

Answer 32

lungs and digestive enzymes (pancreas)

Question 33

CFTR

Answer 33

Cystic Fibrosis Transmembrane Conductance Regulator

Question 34

Individuals who inherit only one faulty gene are called as

Answer 34

"carriers"

Question 35

most common in what decent?

Answer 35

Caucasians of Northern Europe Descent

Question 36

digital clubbing is a sign of

Answer 36

hypoxia (low oxygen levels)

Question 37

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

Answer 37

Digital Clubbing

Question 38

is a sinonasal manifestation of cystic fibrosis

Answer 38

Nasal Polyps

Question 39

Signs and Symptoms of Cystic Fibrosis:

Answer 39

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

Question 40

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

Answer 40

nebulized mist

Question 41

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

Answer 41

A mucolytic

Question 42

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

Answer 42

Chest Physiotherapy

Question 43

How to diagnose Cystic Fibrosis

Answer 43

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

Question 44

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

Answer 44

pancreatic enzyme replacement therapy (PERT)

Question 45

PERT involves taking

Answer 45

oral pancreatic enzyme supplements with meals and snacks

Question 46

elevated blood pressure in the lungs,

Answer 46

pulmonary hypertension.

Question 47

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

Answer 47

bronchiectasis

Question 48

Nursing Diagnosis for Cystic Fibrosis

Answer 48

*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

Question 49

________ of the chest becomes enlarged in CF

Answer 49

The anteroposterior diameter of the chest