Pediatric Pathophysiology

Question 1

Who falls into “Pediatric”?

Answer 1

Newborn: up to 4 weeks of age (28 days)

Infant: 30 days to 1 year old

Child: 1 to 8 to 12 years

Teen: 13 to 18 years

Question 2

Special Considerations of Pediatric Population

Answer 2

• Age and ability to comprehend
  
  • Keep teaching/learning short
  • Use fun games approach if applicable
  • Enlist family assistance
• Parents present or absent
  
  • Decision maker for the child, depending on age
  • Parents to be contacted when treatment discussions are reviewed with the child
• Emotional State
  
  • Frightened, confused, angry, sad.
  • Comfort them
  • Have toys and friendly objects present
• Physical limitations
  
  • Certain diagnostic tests can be dependant on age (PFTs)
• Presentation
  
  • Inability to properly express themselves

Question 3

Pediatric Special Consideration

Presentation

Answer 3

Prenatal and birth Hx

Developmental Hx

Social Hx of family

Immunization Record

Parent as historian

Question 4

What makes a pediatric patient different?

Answer 4

They are NOT little adults!

Most therapies, interventions, and drug regimens are not geared, tested, or approved for their population

Pharmacology

Question 5

Pediatric signs of increased WOB

Answer 5

Tachypnea

Apnea

Nasal Flaring

Grunting

Head Bobbing

Seesaw Respirations

Intercostal Retractions

Tracheal Tug

Question 6

Preventable Pediatric Disorders

Answer 6

Abuse

MVA’s

Near Drowning

Fires

Question 7

Acute epiglottitis Vs Laryngotracheobronchitis(croup)

CXR

Answer 7

Croup- Subglottic narrowing, lower in the airway

Epiglottis-Swollen epiglottis (thumb sign), higher in the airway

Question 8

Acute epiglottitis Vs Laryngotracheobronchitis(croup)

Symptons

Answer 8

Croup- Stridor, barking cough, fever variable, hoarse voice, no position preferred, retractions, irritable, does not appear acutely ill

Epiglottis-stridor, minimal cough, high feer, muffled voice, prefers sitting upright with chin forward, rertactions, drooling, anxiety, appear acuetly ill

Question 9

Acute epiglottitis Vs Laryngotracheobronchitis(croup)

History

Answer 9

Croup- Gradual onset (2-3 days), previous cold

Epiglottis-Acute onset (few hours), compliant of sore throat

Question 10

Acute epiglottitis Vs Laryngotracheobronchitis(croup)

Cause

Answer 10

Croup- Viral (parainfluenza, RSV)

Epiglottis-Bacterial (harmophilius influenze type B)

Question 11

Acute epiglottitis Vs Laryngotracheobronchitis(croup)

Age

Answer 11

Croup- 3 mon to 3 yr

Epiglottis-2-6 yr

Question 12

Bronchiectasis

Answer 12

• Irreversible dilation and distortion of the bronchial tree
• Develops as a result of airway obstruction, chronic infection and extensive inflammation
  
  • Dilation due to destruction of bronchial cartilageelastic fibres, blood vessels and smooth muscle
  • Foreign body aspiration, measles, pertussis, pneumonia, CF
  • Congenital condition in a small number of patients

Question 13

Bronchiectasis

Pathophysiology

Answer 13

Irregularly shaped and dilated bronchi

leads to insufficient airway secretion clearance

Inadequate ciliary activity

The poor bronchial toilet increases the risk of infection

Question 14

Bronchiectasis

Clinical Manifestations

Answer 14

Chronic, productive cough

Copious amounts of thick, purulent sputum

Foul smelling breath

Multiple URIs

SOBOE

Hemoptysis on occasion

Question 15

Bronchiectasis

Management

Answer 15

Supplemental oxygen

Typically diagnosed by clinical manifestations and bronchoscopy or CT scan

Chest physiotherapy and postural drainage

Abx based on sputum culture

Surgical resection of the broncheictaticsection of the lung may be considered

Depends on severity and if the disease if localized

Question 16

Bronchiolitis

Answer 16

• Inflammatory disease of the bronchioles
• Most common lower respiratory infection in pediatrics
• Viral infection of small airways
• Most common cause: RSV (Respiratory Syncytial Virus)

Question 17

Bronchiolitis Pathophysiology

Answer 17

• Airway wall inflammation and edema, ++ increased mucous production
• Small airwayobstruction
• Primarily effects pediatrics <2
• Infants prone to Respiratory Failure from Acute Bronchiolitis:
  
  • Immunodeficientinfants, comorbidities of CHD, CF, childhood asthma

Question 18

Bronchiolitis Causes

Answer 18

RSV (Respiratory Syncytial Virus)

• Approx. 90% children infected in first 2 years of life, mostly presents as cold like symptoms
• RSV diagnosed by Nasopharyngeal swab or aspirate
• Predictable outbreaks from January to April

Question 19

Bronchiolitis Clinical Manifestation

Answer 19

Productive cough

Resp. distress: wheezing, tachypnea, accessory muscle use, nasal flaring, hypoxemia, ++ secretions

O/A coarse crackles

Hyperinflation on CXR

High PaCO2 (in severe cases)

Usually Clinical Diagnosis (by symptom presentation)

75% of infants after acute bronchiolitis have recurrent episodes of coughing and wheezing (often diagnosed as infant asthma)

Question 20

Bronchiolitis Treatment

Answer 20

• Supportive in the majority of cases:
  
  • Bronchodilaters
  • Racemic epinephrine for severe bronchoconstriction
  • RSV-specificimmunoglobintherapy
• Severe Cases:
  
  • CPAP
  • HFNC
  • May require intubation

Question 21

Bronchiolitis CXR

Answer 21

Reveal flattening of the diaphragm and widening of the anteroposterior diameter, which is indicative of severe air trapping.

Perihilar markings accentuated.

Question 22

Cystic Fibrosis (CF)

Answer 22

Recessive exocrine gland disorder characterized by excessive viscid secretion

Question 23

Cystic Fibrosis (CF)

Pathophysiology

Answer 23

• Most common fatal geneticdisease affecting Canadian children and young adults
• Caused by mutations in the gene that encodes multifunctional protein: CF transmembrane conductaneregulator (CFTR)
  
  • Function of this protein is to serve as an apical (side toward the lumen) chloride channel in:
    
    • Airway cells-causing improper regulation of sodium-chloride ions leading to thick, tenacious secretions
    • Intestinal cells
    • Exocrine glands
• Variability of disease symptoms caused by over 1900 types of CFTR mutations

Question 24

Cystic Fibrosis (CF)

Clinical Manifestation

Answer 24

• Dry cough progressing to frequent wet coughing spells with large quantities of secretions
• Anorexia, weight loss
• Wheezing
• SOBOE
• Chronic colonization of P. Aerguinosa

Question 25

Cystic Fibrosis (CF) Cycle of Infection

Answer 25

* CFTR dysfunction leads to a decrease in airway surface liquid which impairs mucous clearance, which leads to airway inflammation/infection. This results in chronic productive cough and Hyperinflation/Bronchiectasis. * Endstage CF: * pulmonary HTN * CorPulmonale * Most common pathogens associated with CF: * Staph Aureous, * Haemophilus influenza, * Pseudomonas * Aeruginosa

Question 26

Cystic Fibrosis (CF) Non-Respiratory Manifestations

Answer 26

* Pancreatic Insufficiency * Malabsorption of fats and proteins * Failure to thrive in infants * Meconium ileus (infants) * Meconium but on a much larger scale to the point where their ability to expel the meconium has been blocked * Reduced fertility * Increased risk of Diabetes

Question 27

Cystic Fibrosis (CF) Diagnosis

Answer 27

* Sweat Chloride Test (\> 60 mmol/L) * Sodium Chloride concentration in sweat are abnormally high in CF * A small amount of a colorless odorless sweat producing chemical is applied to the patient’s arm. An electrode is attached to the chemically prepared area, and a mild electric current is applied to stimulate sweat production. * Infant Screening * Failure to gain weight * Bronchiectasis * Recurrent Pancreatitis

Question 28

Sweat Chloride Test Infants Six Months or Younger

Answer 28

≤29 mmol/L: Normal (CF very unlikely) 30 to 59 mmol/L: Intermediate (Possible CF) ≥60 mmol/L: Abnormal (CF Diagnosis)

Question 29

Sweat Chloride Test Infants Older than Six Months, Children and Adults

Answer 29

≤39 mmol/L: Normal (CF very unlikely) 40 to 59 mmol/L: Intermediate (Possible CF) ≥60 mmol/L: Abnormal (CF Diagnosis)

Question 30

Monitoring at a CF Clinic

Answer 30

Symptom assessment Physical examinations Regular sputum cultures Spirometry Nutritional Status

Question 31

Types of Treatment for CF

Answer 31

Bronchopulmonary Hygiene Medications Lung Transplant

Question 32

CF Treatment Bronchopulmonary Hygiene

Answer 32

* Percussion * Postural Drainage * BIPAP (during physical therapy) Autogenic Drainage

Question 33

CF Treatment Medications

Answer 33

Bronchodilators Inhaled Recombinant Deoxyribonuclease(DNase)(Pulmozyme)–daily in \>6 yrs Nebulized 7% hypertonic saline (BID in patients \>6 yrs) help draw out secretions N-acetyl-cysteine (Mucomyst) – Help pplcough up secretion s Ibubrofen Azithromycin (patients \> 6 yrs) Tobramycin (treat P. Aeruginosa)

Question 34

Where do RT fit in with CF Treatment

Answer 34

* End stagemanagement –BIPAP * Post-op Lung Transplant management * Home Oxygen Therapy * Spirometry-CF clinics GOOD NEWS! Due to improvements in therapy, median age of survival has increased from 31 years to 41 years in past two decades