T3-Blueprint: CF and Celiac Flashcards Preview

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Flashcards in T3-Blueprint: CF and Celiac Deck (37):
1

Exocrine gland dysfunction leading to increased mucous production

CF

2

CF: Mucus glands secrete thick _____ that accumulates and ___ the gland

Secrete thick MUCOPROTEIN that accumulates and DILATES the glands

3

CF: Small passages in organs (especially ____ & ____) become obstructed when secretions coagulate and become hard in the glands and ducts, leading to fibrosis and failure

Pancreas and bronchioles

4

Results from sensitivity to gluten

Celiac

5

What is gluten?

Protein found in grains

6

Celiac: Vili of the small intestine _____, reducing the absorptive surface leading to decreased digestion of ___ and decreased absorption of ___ & ___

Villi of small intestine FLATTEN out, reducing the absorptive surface leading to decreased digestion of FAT and decreased absorption of VITAMINS AND CARBS

7

What are the early respiratory S/S of CF?

Wheezing
Dry, non-productive cough

8

What are the late respiratpory signs of CF?

-Dyspnea
-Paroxysmal cough
-Obstructive emphysema
-Patchy areas of atelectasis
-Frequenty pulmonary infections
-Overinfalted, barrel shaped chest
-Cyanosis
-Clubbing of fingers and toes

9

What are the early GI s/s of CF?

Meconium ileus

10

What are the late GI s/s of CF?

-Large, bulky, frothy, foul-smelling stools
-Increased appetite early in disease (with no weight gain)
-Decreased appetite later--> failure to grow and anemia
-Fat soluble vit. deficiency
-Thin extremities
-Tissue wasting

11

What are s/s of celiac disease?

-Irritability
-Anorexia
-Chronic diarrhea
-Steatorrhea
-Vit. deficiencies
-Abdominal distention
-Malnutrition
-FTT

12

How do we test for celiac?

Biopsy of small intestine

13

How do we test for CF?

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

14

CF: What is a normal sweat chloride test?

Less than 40 mEq/L

15

CF: What is the level for infants with CF who had sweat chloride test?

Greater than 60 mEq/L

16

CF: What is the level for all other ages with CF who had a sweat chloride test?

Greater than 40 mEq/L

17

What do stools look like for CF patients?

-Frothy
-Foul smelling
-Fat containing
-Float (due to fat content)

18

What are the fat soluble vitamins?

A, D, E, K

*CF patients have fat containing stools

19

One of the early signs for CF is meconium ileum. What is this?

Bowel obstruction

20

CF: Describe meconium ileus characteristics.

Thick, puttylike, tenacious, mucilaginous meconium that blocks the lumen of the small intestine

21

CF: Meconium ileum blocks the lumen of the small intestine. Where does this usually happen and what does that mean?

Usually at or near the ileocecal valve, which gives rise to intestinal obstruction (abdominal distention, vomiting, failure to pass stools, rapid dehydration with electrolyte imbalance)

22

What is the diet for CF?

High protein, high calorie

23

What is the diet for celiac?

Rice, corn, soybean flours

24

T/F: It is okay to give a child with celiac disease foods with wheat, barley, rye, or oats

NO

25

What respiratory meds do we give for CF?

-SABA: albuterol
-Anticholinergics: Ipratroprium
-Combo: fluticasone/salmaterol
-Human deoxyribonuclease
-Dornase alfa (decreases viscosity of mucous)

26

What are some antibiotics we give CF patients?

Tobramycin
Tircarcillin
Gentamycin

27

What GI meds do we give CF patients?

-Pancreatic enzymes
-Vitamines: multi and ADEK
-Constipation meds: Polyethylene glycol (golytely)
-H2 receptor agonist and motility meds for GERD

28

Does the US have universal newborn screening for CF?

No, only 2 states offer this

29

CF Screening: Children who were identified and treated early in infancy with aggressive nutritional support had improved ___ & ___ well into adolescence

Height and weight

30

If a newborn were to be screened for CF, how is it done?

Immunoreactive trypsinogen analysis performed on a dried sport of blood...which may be followed by direct analysis of DNA for presence of mutations in the same dried spot of blood

31

CF: Prevention of pulmonary infection involves a daily routine of CPT to maintain pulmonary hygiene. What is CPT?

Patient is dressed in a light shirt and placed in a postural drainage position. The practitioner gently but firmly strikes the chest wall with a cupped hand. A popping, hollow sound, NOT a slapping sound, should be the result. This procedure is done over the RIBCAGE ONLY

32

How often is CPT done on CF patients?

On average twice failing, on rising and in the evening

*and more frequently during pulmonary infection

33

CF: A small, handheld plastic pipe with a stainless steel ball on the inside that facilitates removal of mucus

Flutter Mucus clearance device

34

What are the advantages of the flutter mucus clearance device?

Increasing septum expectoration and being used without assistance

35

CF: What is the cornerstone of pulmonary therapy?

CPT---CF patients have been found to REGRESS when CPT is discontinued!--although it is time consuming for child and family, it is the cornerstone treatment

36

The term celiac disease is often used to describe a symptom complex with 4 characteristics. What are those 4 characteristics?

1. Steatorrhea
2. General malnutrition
3. Abdominal distention
4. Secondary vitamin deficiencies

37

Celiac: What does steatorrhea mean?

Fat, frothy, foul, bulky stool

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