Respiratory Part 2 - Pediatrics

Front 1

What is Cystic Fibrosis (CF)?

Back 1

A common inherited autosomal recessive disorder of the exocrine glands (mucous producing) that results in physiologic alteration in the respiratory, gastrointestinal, integumentary, musculoskeletal, and reproductive systems. Abnormal mucus secretion and obstruction. It is progressive and incurable.

Front 2

What are common symptoms associated with Cystic Fibrosis?

Back 2

Pancreatic enzyme deficiency and pancreatic fibrosis caused by duct blockage (85% of pt's), progressive chronic lung disease as a result of infection, and sweat gland dysfunction resulting in ↑ Na and Cl sweat concentration.

Front 3

What is a quantitive sweat chloride test?

Back 3

1. Production of sweat is stimulated.
2. Sweat is collected and measured (more than 75mg).

Normal lab value for sweat chloride concentation is < 40 mEq/L.

Front 4

What is the lab value for a positive sweat chloride test result in infants younger than 3 months?

Back 4

>40 mEq/L

Front 5

What is the lab value for a positive sweat chloride test result in anyone older than 3 months?

Back 5

>60 mEq/L

Front 6

Should sweat chloride results between 40 to 60 mEq/L require a repeat test?

Back 6

Yes. This is highly suggestive of cystic fibrosis.

Front 7

What is the most common cause of death in a patient with Cystic Fibrosis?

Back 7

Respiratory failure.

Front 8

What is Cystic Fibrosis's effect on the respiratory system?

Back 8

Mucus build-up in the lungs lead to bacterial infection and destruction of lung tissue.
Emphysema and atelectasis.
Chronic hypoxemia leads to pulmonary htn and eventual cor pulmonale.
Pneumothorax and hemoptysis.

Front 9

When do respiratory symptoms usually occur in Cystic Fibrosis pt's?

Back 9

Usually before 1 year old

Front 10

What are other respiratory symptoms in Cystic Fibrosis?

Back 10

Weezing and cough
Dyspnea
Cyanosis
Clubbing of the fingers and toes
Barrel chest
Repeated episodes of bronchitis and pneumonia.

Front 11

What is the earliest manifestation of Cystic Fibrosis?

Back 11

Meconium ileus

Front 12

What effects does Cystic Fibrosis have on the GI system?

Back 12

Intestinal obstruction (S/S pain, abd distention, N/V)
Frothy, foul smelling stools (fatty stools)
Deficiency of the fat-soluable vitamins A,D,E, and K (can result in easy bruising, bleeding, and anemia).
Malnutrition and failure to thrive (ftt) is a concern.
Rectal prolapse can result from lrg bulky stool.
Pancreatic fibrosis can occur and places the child at risk for DM.

Front 13

What effects does Cystic Fibrosis have on the integumentary system?

Back 13

Abnormally high concentration of NA and Cl in sweat.
Parents report infant tastes "salty" when kissed.
Dehydration and electrolyte imbalances can occur.

Front 14

What effects does Cystic Fibrosis have on the reproductive system?

Back 14

May delay puberty in girls.
Mucus cervical secretions may block sperm entry.
Males are usually sterile

Front 15

What diagnostic tests are needed for pt's c Cystic Fibrosis?

Back 15

Sweat Chloride test (increased electrolyte), Chest x-ray (patchy atelectasis and obstructive emphysema), and Stool for fat or enzyme analysis.

Front 16

What is chest physiotherapy (CPT)?

Back 16

Percussion and postural drainage

Front 17

When should CPT be done?

Back 17

On awakening and in the evening qd.
CPT should not be performed before or immediately after a meal (severe coughing may lead to vomiting).
CPT should be done more frequently during pulmonary infection.

Front 18

What should be administered prior to CPT?

Back 18

Bronchodilator if pt has reactive airway disease or is wheezing.

Front 19

What does clinical therapy focus on for pt's c Cystic Fibrosis?

Back 19

Maintaining respiratory function, managing infection, promoting optimal nutrition and exercise, and preventing GI blockage.

Front 20

When should Pancreatic enzymes be given?

Back 20

With meals taken as whole capsule or sprinkled on food.

Front 21

Why is a diet high in protein and carbohydrate, up to 150% of recommended daily allowance, needed in patients with Cystic Fibrosis?

Back 21

To prevent wasting due to malnutrition as a result of malabsorption.

Front 22

What is pneumonia?

Back 22

Inflammation of the pulmonary parenchyma or alveoli or both cuased by a virus, mycoplasmal agents, bacteria, or aspiration of foreingn substances.

Front 23

What are S/S of pneumonia?

Back 23

SOB, productive cough, fever, cyanosis, decreased SaO2, crackles, increased RR, increased Pulse, dyspnea, nasal flaring, rhonchi, wheezes. Decreased breath sounds may be present if consolidation exists.

Front 24

How do you treat pneumonia?

Back 24

Depends on the causative agent.
Symptomatic therapy: pain and fever control.
Supportive care: Airway management, fluids and rest.
Antibiotics if bacterial in nature.

Front 25

How is pneumonia diagnosed?

Back 25

CXRAY

Front 26

Clinical manifestations of asthma:

Back 26

Respirations are rapid and labored because of ongoing exertion of breathing. Inspiratory and expiratory wheezing, use of accessory muscles, decreased air movement, & fatigue.

Front 27

What are triggers in asthma?

Back 27

Exercise, pollens, pollutants, dust, molds, dust mites, cockroach feces, whether changes, or emotions.

***NOTE: Viral illness is most common trigger.

Front 28

Diagnostic Tests & Medical Management for Asthma:

Back 28

Confirmed by pulmonary function tests.
Peak Flow (green 80-100%, yellow 50-80%, red <50%)
Bronchodialators
Steroids
Leukotrene Inhibitors