T3 - Cystic Fibrosis and Celiac Disease Flashcards Preview

Peds (Josh) > T3 - Cystic Fibrosis and Celiac Disease > Flashcards

Flashcards in T3 - Cystic Fibrosis and Celiac Disease Deck (29):
1

CF is characterized by --- gland dysfunction.

exocrine (mucus-producing)

2

T/F: CF is inherited.

True

3

CF is seen primarily in --

white children (male and female)

4

Where is the location of the mutated gene w/ CF?

long arm of chromosome 7

5

What number of the population are carriers for CF?

1 in 25 caucasions

6

With each pregnancy, two carrier parents have a -- chance of having a child w/ CF.

1:4

- there is a 50% chance child will be a carrier

- there is a 25% chance child will neither have CF or be a carrier

7

How CF is passed from parents. 

see the pic in ppt

8

A -- -- is useful for calculating the chances of having a child w/ CF. 

Punnett Square

9

Patho of CF

Mucous glands secrete thick mucoprotein that accumulates and dilates the glands.

Small passages in organs (esp. pancreas and bronchioles) become obstructed when secretions coagulate and become hard in the glands and ducts leading to fibrosis and failure.

10

How does CF affect the GI tract?

impairs absorption of nutrients (fat-soluble vitamin absorption is decreased)

11

CF leads to what type of stools?

frothy stools from undigested fat

12

What else occurs in GI tract due to CF?

Isles of Langerhans decreases

Fibrosis in liver and Biliary obstruction

Prolapse of rectum

Intestinal obstruction

13

S/S of CF

Short Stature

Sinusitis

FTT

Nonproductive Cough

Increased AP chest diameter

Protuberant abdomen

Digital Clubbing

14

What would CF stool's look like (ie: the Four F's)?

Frothy (bulky and large quantity)

Foul Smelling

Fat Containing

Float (due to fat content)

15

What type of tests are used to measure for CF?

two positive SWEAT TESTS

- presence of high NaCl concentrations in sweat is a unique characteristic of CF

16

Parents will sometime say their child tastes --- with CF.

salty

- presence of high NaCl concentrations in sweat is a unique characteristic of CF

17

Early Signs of CF in airway

Wheezing

Dry, nonproductive cough

18

Late signs of CF in airway

dyspnea

paroxysmal cough

obstructive

emphysema’patchy areas of atelactasis

frequent pulmonary infections

overinflated, barrel-shaped chest

cyanosis

clubbing of fingers and toes

19

Early sign of CF in GI tract

meconium ileus

20

Late signs of CF in GI tract

large, bulky frothy, foul-smelling stools

increased appetite early in disease (w/ no weight gain)

decreased appetite--later

failure to grow and anemia

fat soluble vitamin deficiency

thin extremities and tissue wasting

21

Which type of diet for CF?

high protein, high calorie

- may need up to 150% of daily requirements

22

What is Flutter Therapy for CF?

Looks similar to a fat pipe that has a steel ball encased in it.

The patient blows on the pipe, the ball vibrates and sends the vibrations down the airways and into the lungs.

The vibrations loosen the mucus and help it move up the airways.

Works 3 x better than chest physiotherapy.

23

-- is an enzyme that chops up DNA and causes liquefied form of mucous (which is easier to cough out).

Pulmonzyme

24

Celiac Disease results from what?

sensitivity to gluten (protein found in grains)

25

In CD, the villi of the small intestine do what?

flatten out

- reduces absorptive surface
- reduces fat digestion
- reduces absorption of vitamins and carbs

26

CD clients first present symptoms that look like --

CF

27

Digestive disturbances from CD usually seen at what age?

6-12 mths

28

S/S of Celiac's Disease

Irritability

Anorexia

Chronic diarrhea

Steatorrhea

Vit deficiencies

Abdominal distention

Malnutrition

FTT

29

CD clients can eat ---

CD clients cannot eat ---

Can eat grains (rice, corn, soybean flours)

Cannot eat wheat, barley, rye, oats