Quiz Pedi

Cystic Fibrosis (Mucoviscidosis)

Definition: An inherited, genetic disorder involving the exocrine glands. Results in thick viscous

mucus accumulation in the lungs, blocks passageways of the pancreas, and prohibits enzymes

from reaching the intestines leading to inhibition of digestion of protein and fat and deficiencies

of vitamins A, D, E and K

Patient Assessment:

Past Medical History

failure to thrive

Positive family history, meconium ileus as newborn, recurrent respiratory infections,

General Appearance

Barrel chest, cyanosis, clubbing, small for age, malnutrition, poor body development,

peripheral edema

Respiratory Pattern

Tachypnea, dyspnea on exertion, pursed lip breathing, use of accessory muscles of

inspiration and expiration, cough productive of large amount of thick purulent secretions

Breath Sounds

Diminished, crackles, wheezing

Diagnostic Chest Percussions: Hyperresonant or tympanic note

Diagnostic Testing:

Chest X-ray

Translucent (dark) lung fields, depressed or flattened diaphragm, right ventricular

enlargement, areas of atelectasis and fibrosis

Arterial Blood Gas

Mild to moderate stages: Acute alveolar hyperventilation with hypoxemia

Severe stages: Chronic ventilatory failure with hypoxemia

Pulmonary Function:

Decreased flowrates (FEV1, FEF 25-75, FEF 200-1200)

CBC: Elevated Hb and Hct concentrationSputum Culture:

aeruginosa

Often positive for Staphylococcus aureus, Haemophilus influenzae, Pseudomonas

Special Tests:

Newborn screening by immunoreactive trypsin level (IRT) required in all 50 states

Sweat Chloride Test (chloride level > 60 mEq/L)

Genetic Testing of CFTR mutation

Treatment/ Management

A. Airway Clearance

Chest percussion and postural drainage

Exercise

PEP therapy

High frequency chest wall compression devices

Forced expiration techniques

Active cycle of breathing

Autogenic drainage

Huff coughing

B. Oxygen therapy as needed

C. Drug therapy

a. Aerosol theory

i. Bronchodilator

ii. Mucolytics - dornase alpha (Pulmozyme)

iii. Corticosteroids

1. Advair

2. Flovent, Pulmicort

iv. Inhaled Antibiotics

1. Tobramycin (TOBI)

2. Colistin

3. Amikacin

v. Digestive enzymes

Pedi Asthma (Reversible Airway Obstruction)

Common triggers include allergies, stress, exercise, cold exposure, infection and inhaled

irritants

Clinical Presentation/Diagnosis

In early stages: dyspnea, cough, increased production of secretions, accessory

muscle use, increased respiratory rate and expiratory wheezing

Young children may complain of a stomach-ache-

Parents may notice lethargy and decreased play activity

During an acute episode, arterial blood gases may show hypoxemia with or

without hypercarbia and the chest X-ray may show hyperinflation, flattened

diaphragms and infiltraties

A patient with decreased breath sounds (silent chest) and then increased

wheezing indicates improved air movement

Helpful diagnostic tests include:

Pulmonary function testing (spirometry)

Exhaled nitric oxide testing (Feno) helpful in monitoring airway

inflammation

Bronchoprovocation challenges with methacholine or exercise

Goal of Asthma Treatment is to control symptoms

A. Control medications

a. Long acting beta1 agonists (LABA) : Salmeterol, formoterol, arformoterol

b. Inhaled corticosteroids: beclomethasone, budesonide, fluticasone

c. Leukotriene modifiers: montelukast, zileuton

d. Immunomodulators: omalizumab, palivizumab

B. Quick relief (rescue) medications

a. Short acting beta 2 agonists (SABA) : Albuterol, levalbuterol

b. Anticholinergics: ipratropium

c. Systemic corticosteroids: prednisone, methylprednisolone

C. Emergency room care

a. Start with oxygen therapy

b. Inhaled SABA agents: 3 treatments/ hour or continuous

c. Inhaled anticholinergics

d. Systemic corticosteroids

Status asthmaticus is an acute episode that fails to respond to usual bronchodilator treatment

a. Additional treatment may consist of:

i. Continuous aerosol bronchodilator

ii. Subcutaneous epinephrine

iii. Intravenous steroids

iv. Magnesium sulfate

v. He/O2 therapy

vi. Inhaled anesthetics (isoflurane, sevoflurane, halothane)

b. Mechanical ventilation may be indicated for rising PaCO2 increasing spontaneous

minute ventilation, or decreased level of consciousness

i. Paralyze and sedate the patient as needed

ii. If possible, attempt non-invasive ventilation, first to avoid intubation

iii. Use low respiratory rate with long expiratory time

iv. Consider permissive hypercapniaPeak flow monitoring essential to monitoring and managing asthma

Recommend comprehensive asthma management program which includes:

A. Patient and parent education

B. Identification and avoidance/ management of triggers

C. Peak flow monitoring

D. Recognizing signs and symptoms of episodes

E. Asthma action plan

Pedi Infectious Disease/ Pedi Pneumonia

Lower respiratory tract infections are a frequent cause of morbidity and mortality in children,

especially those younger than 2 years of age

Causative Agents

A. Viral Pneumonia - most common type of pneumonia

a. Symptoms include runny nose (coryza) , nasal congestion, cough, fever, malaise

b. Respiratory Syncytial Virus (RSV) - most common cause of viral pneumonia

i. Occurs most comonly during the winter months (from mid- December

through March)

ii. Chest X-ray reveals hyperinflated lungs with patchy infiltration and/or

atelectasis

iii. Diagnosis confirmed by rapid immunofluroscent detection of RSV antigen

in nasal washings

iv. Previously treated with aerosalized ribavirin (Virazole) administered by

small particles aerosol generator (SPAG)

v. RSV is also responsible for the majority of cases of bronchiolitis in young

children

c. Other causative agents

i. Parainfluenza Virus Types 1,2,3

ii. Influenza Virus

iii. Adenovirus

iv. Enterovirus

v. Coronavirus

Bacterial Pneumonia

a. Higher mortality rate than viral pneumonia

b. Risk factors include immunocompromise, aspiration from GERD, malnutrition, day care

or school attendance

c. Signs and symptoms similar to viral pneumonia

d. Causative agents

i. Neonatal patients: Group B streptococcus, Escherichia coli.

ii. Pediatric patients: Staphylococcus pneumoniae, H influenza, S aureuse. Diagnosis aided by blood cultures and elevated band counts (>1500 total bands)

f. Treatment includes antibiotic therapy

Supportive Care Includes

a. Oxygen therapy as indicated by oximetry

b. Fluid management

c. Nutritional support

d. Trial of bronchodilators may be helpful

Croup (Laryngotracheobronchitis)

Definition: An inflammatory process that causes edema and swelling of the mucous

membranes just below the vocal cords (subglottic area) resulting in airway obstruction

Etiology: Primarily a viral infection, most often causes by Parainfluenza viruses or RSV

transmitted by aerosol droplets

Patient Assessment:

Patient Medical History: Recent cold that developed gradually into a barking cough over 2-3

days, more common in the fall and winter

Cough: Characteristic barking cough, stridor, hoarse voice

Physical Appearance: Cyanosis, alert with some accessory muscle use, nasal flaring, rhinorrhea

Breath Sounds: Diminished with inspiratory stridor

Vital Signs: Increased HR, BP, low grade fever

Diagnostic Testing

a. Lateral Neck X-ray: Haziness in the subglottic area, steeple sign, pencil point, picket

fence, hour-glass narrowing of the upper airway

b. Arterial Blood Gas: Acute alveolar hyperventilation with hypoxemia

Treatment/ Management

a. Mild Cases

i. Supportive care

1. Temperature control - cool environment

2. Adequate hydration and humidification of inspired air3. Closely monitor

a. Vital signs, including pulse oximetry

b. Degree of retractions

c. Level of consciousness

ii. Oxygen therapy 30-40%

iii. Cool aerosol mist ( face mask)

iv. Drug therapy

1. Racemic Epinephrine (MicroNefrin, Vaponefrin)

2. Corticosteroids - For children who do not respond to cool aerosol and

racemic epinephrine therapy

b. Severe Cases

i. Child with severe respiratory distress and/or marked inspiratory stridor

1. Criteria for intubation

a. Lethargic, exhausted

b. Severe stridor at rest

c. Diminished breath sounds

d. Extreme accessory muscle usage

2. Temperature control - cool environment

3. Adequate hydration and humidification of inspired air

4. Transfer patient to ICU

5. Sedate to prevent inadvertent extubation

6. Place on T-piece or CPAP

7. Criteria for extubation

a. Child's condition is stable

b. Air leak around the tube (swelling has gone down)

Epiglottitis

Definition: Life threatening emergency caused by inflammation of the supraglottic region that

includes the epiglottis, aryepiglottic folds, and false vocal cords that causes swelling just above

the vocal cords

Etiology: Bacterial infection caused by Haemophilus influenza B (gram negative bacteria)

transmitted by aerosol droplets

Patient Assessment:

Past Medical History: Sudden onset within 6-8 hours

Cough: Muffled coughPhysical Appearance: 2-6 years of age, pale or cyanotic, lifeless, drooling, hoarseness, difficulty

swallowing (dysphagia), tongue thrusts forward during inspiration, voice and cry muffled, jaw

jutted forward

Respiratory Pattern: Tachypnea, nasal flaring, substernal and intercostal retractions

Breath Sounds: Diminished with inspiratory stridor

Vital Signs: Increased HR, BP, high grade fever

Diagnostic Testing

Avoid any unnecessary stimulation of the child

Lateral Neck X-ray: Haziness in the supraglottic area (epiglottis), supraglottic swelling (above

the glottis) or “Thumb Sign”

Arterial Blood Gas: Acute alveolar hyperventilation with hypoxemia

CBC: Elevated WBC

Treatment/Management

a. Immediate placement of an artificial airway

i. Endotracheal tube

ii. Tracheostomy if unable to intubate

b. Transfer patient to ICU

c. Sedate to prevent inadvertent extubation

d. Place on T-piece or CPAP

e. Oxygen therapy

f. Drug therapy

i. Antibiotics

g. Criteria for Extubation

i. Child's condition is stable

ii. Swelling in the airway has diminished

Bronchiolitis/ RSV

Definition:

a. Acute infection of the lower respiratory tract, usually caused by the respiratory syncytial

virus (RSV)b. c. rESULTS IN INFLAMMATION AND OBSTRUCTION OF THE SMALL BRONCHI AND

BRONCHIOLES AND EXCESSIVE AIRWAY SECRETIONS

Patients at risk include children less than 1 year of age, children with weakened immune

systems, and children with chronic respiratory or cardiac disease

Patient Assessment

Past Medical History: Upper respiratory infection in young children

General Appearance: Nasal discharge, lethargic, nasal flaring, cyanosis

Respiratory Pattern: Tachypnea, apnea in severe cases, grunting, intercostal and substernal

retractions, intermittent cough

Breath Sounds: Wheezes, crackles, upper airway noise from secretions

Diagnostic Chest Percussion: Hyperresonance in severe cases

Diagnostic Testing

Chest X-ray: Hyperinflation with areas of consolidation or atelectasis

Arterial Blood Gas: Acute alveolar hyperventilation with hypoxemia

Pulmonary Function: Decreased flowrates (FEV1, FEF 25-75%, and FEF 200-1200)

Special Tests: Detection of RSV antigen in washings from nasopharynx or oropharynx by

1. 2. RSV- enzyme immunoassay (EIA) or

Respiratory infectious disease panel (RIDP) by polymerase chain reaction (PCR)

Treatment/ Management

A. B. C. Prophylaxis with respiratory syncytial virus immune globin (RespiGam) or palivizumab

(Synagis) recommended for children at risk

Many children can be treated at home with humidification and oral decongestants

For children admitted to the hospital

a. Systemic hydration

b. Oxygen Therapy to maintain acceptable SpO2

c. Bronchodilators to relieve wheezing

d. Airway clearance therapy

e. Mechanical ventilation for acute ventilatory failureBronchopulmonary Dysplasia (BPD)

1. This is a chronic condition that results from treatment of RDS with mechanical ventilation and

high concentrations of oxygen over a prolonged period of time ( >28 days)

2. Diagnosis of BPD is made in newborns who are oxygen- dependent and have an abnormal

chest X-ray after 28 days of age

3. Clinical Presentation/ Diagnosis

a. b. c. Tachypnea, retractions and persistent cyanosis

Lengthy ventilatory course ( > 14 days) associated with poor response to therapy

X- ray shows small areas of lucency alternating with areas of irregular densities

4. Prevention

a. Lung-protection strategies when mechanical ventilation is required

b. Titrate FiO2 to use lowest level possible

c. Proper nutritional therapy

d. Corticosteroids

5. Treatment

a. Supplemental low flow oxygen therapy as required

b. Careful fluid management

c. Diuretics to reduce lung edema

d. Bronchopulmonary hygiene

e. Bronchodilators

Congenital Heart Defects

Definition: Structural abnormalities of the heart present at birth. The most serious defects

create right-to-left shunting, resulting in severe hypoxemia. These include:

a. b. Tetralogy of Fallot: Overriding aorta, pulmonary stenosis, ventricular septal defect, and

right ventricular hypertrophy

Transposition of the great vessels - aorta is switched with pulmonary artery (aorta arises

from the right ventricle and the pulmonary artery arises from the left ventricle)

Patient Assessmenta. General Appearance : Cyanosis

b. Respiratory Pattern: Tachypnea

c. Auscultation: Normal breath sounds, loud heart murmur

Diagnostic Testing

a. b. c. Chest X-ray - possibly an enlarged or abnormally shaped heart

i. Egg-shaped heart with Transposition of the great vessels

ii. Boot-shaped heart with Tetralogy of Fallot

Echocardiogram is the most important diagnostic test to identify cardiac defects

Pre and Post ductal blood gas studies

i. Pre and Post ductal (right radial artery) PO2 is > 15 torr higher than the

post-ductal (umbilical artery) PO2, then the patient has a right-to-left shunt

ii. Can also be evaluated using two transcutaneous monitors. One placed on the

upper right thorax (pre-ductal) and the other on the lower left thigh or left

abdominal region (post-ductal)

Treatment/ Management

a. b. c. d. Oxygen therapy - maintain PaO2 levels between 50-80 torr

Mechanical ventilation for ventilatory failure

Prostaglandins to maintain patient ductus arterosus

Supportive care prior to surgical c