Respiratory

hour - 3

 Continue

Asthma
Extrathoracic
1) Neonatal respiratory distress
syndrome and its complications
2) Cystic Fibrosis
3) Sickle cell patients: acute chest
syndrome
4) SIDS

Pediatric
Asthma
Epidemiology,
Pathophysiology, and
Initial Evaluation

Overview

Chronic disease of the lower airways,

characterized by reversible airway
obstruction, inflammation, and bronchial
hyper-responsiveness

Leads to recurrent episodes of wheezing,

breathlessness, chest tightness and coughing

Epidemiology

Most frequently encountered pulmonary

disease in children

The lifetime prevalence of asthma in

children is 13%

Prevalence rates are highest among Puerto

Rican and African American children

Among the most common reasons for

hospitalizations in the pediatric practice
(3% of all pediatric admissions in 2004)

Epidemiology

Mortality
Although

mortality rates have fallen,

asthma remains a preventable cause of
death in children

In

2004, the mortality rate from asthma

was 2.5 per 1 million children (186
deaths)

In

general, the rate of death of asthma

is higher in severe, uncontrolled disease

Mortality
Risk

factors for mortality due to asthma

include:
One

or more life threatening exacerbations

of asthma
Severe asthma requiring chronic oral
corticosteroids
Poor control of daily asthma symptoms
Abnormal Forced Expiratory Volume in one
second (FEV )
Frequent ED visits
Low socioeconomic status
Family dysfunction
Patient psychological problems
1

Risk Factors
Factors Influencing the Development and
Expression of Asthma
Genetic
Genes

predisposing to atopy
Genes predisposing to airway hyper
responsiveness
Obesity
Sex
Boys

affected more often than girls prior

to adolescence

Risk Factors

Environmental Risk Factors

Allergens
Indoor Domestic mites, furred
animals (dogs, cats, mice),cockroach
allergens, fungi, molds, yeasts.
Outdoor Pollens, fungi, molds,
yeasts.
Infections (predominantly viral)
Occupational sensitizers
Tobacco
Indoor/Outdoor air pollution
Diet

Pathophysiology
Inherent

to asthma is
airway inflammation,
which is mediated by
a variety of cell
subtypes

Airway

inflammation
results in hyperresponsive airways,
which limits airflow
and causes the
variable clinical
symptoms

Pathophysiology

Atopic asthma
Allergen
IgE

Mucus
secretion

Mast cell

Epithelial cell
injury
Muscle
contraction

Muscle contraction

Recruitment of
leukocytes

Release of
inflammatory
mediators

Acute phase

Late-phase

Mucus
secretion

Pathophysiology

Inhaled allergens are ingested by Antigen

Presenting Cells (APCs) which then present pieces
of the allergen to other immune system cells (TH0
cells)
In asthmatic patients, these TH0 cells transform into
TH2 cells, which activates the humoral immune
system, creating antibodies against the inhaled
allergen
All subsequent times the allergen is inhaled, these
antibodies recognize it and activate the humoral
response
Proinflammatory cytokines (IL-4, IL-5, IL-13)
produced primarily by TH2 cells are believed to
trigger the intense inflammation of allergic asthma
The imbalance between TH1 and TH2 lymphocytes
contributes to chronic inflammatory asthma

Pathophysiology
IgE

mediated early phase is the immediate

response to an allergen, which causes mast cells
and basophils to degranulate, precipitating
bronchospasm as well as the release of
proinflammatory cytokines and chemokines

This

cascade of inflammatory response results in

the subsequent late phase obstruction of air
flow, which occurs 4-12 hours following exposure
to the environmental insult

Pathophysiology

Pathophysiology
Asthma

can also be characterized at the cellular

level by structural alterations in the airway
epithelium
Airway remodeling components:
Mucus

gland hyperplasia
Thickening of the epithelial basement membrane
Fibrotic changes in the sub-basement membrane
Bronchial smooth muscle hypertrophy
Angiogenesis

Pathophysiology

Clinical Aspects
Tools for Diagnosis of Asthma in the
Pediatric Population
Good

History Taking (ASK)

Careful

Physical Examination (LOOK)

Investigations

(PERFORM)

Clinical Aspects
Detailed

Medical History (HPI, PMH,

FMH)
Does

the child cough, wheeze, or develop

chest tightness after exposure to airborne
allergens or irritants e.g. smoke, perfumes,
animal fur?
Does the childs cold frequently go to the
chest or take more than 10 days to
resolve?
Does the child use any medication when
symptoms occur? How often?
Are symptoms relieved when medication is
used?

Clinical Aspects
Physical

Examination

General

attitude and well being

Deformity of the chest
Character of breathing
Thorough auscultation of breath sounds
Signs of any other allergic disorders on the
body
Growth and development status

Clinical Aspects
Signs

and Symptoms of Asthma

Recurrent

Wheeze
Recurrent Cough
Recurrent Breathlessness
Activity Induced Cough/Wheeze
Nocturnal Cough/Breathlessness
Tightness Of Chest
Afebrile episodes
Personal atopy
Family history of atopy or asthma
History of triggers
Seasonal exacerbations
Relief with bronchodilator

Clinical Aspects
Clinical Investigations
Spirometry

2007 Guidelines recommend objective

measurement of pulmonary function as
part of the initial evaluation
Spirometry is a pulmonary function test
that measures the volume of air an
individual inhales or exhales as a function
of time
Should be performed before and after
administration of a short-acting
bronchodilator (12% increase in FEV1
suggests asthma)
LIMITATIONS: Cant be performed on
children less than 6 years old

Clinical Aspects
Baseline chest radiographs

Typically show mild hyperinflation and/or

increased bronchial markings

Peak flow monitoring- may be useful

for patients with moderate to severe
asthma

50-80% of predicted = mild to moderate

<30% of predicted = severe obstruction
LIMITATIONS: heavily dependent on
technique

Differential Diagnosis
A

child presents with wheezing and respiratory

distress:
Intraluminal

inflammation or failure to clear

secretions (bronchiolitis, gastroesophageal reflux
with aspiration, cystic fibrosis, tracheoesophageal
fistula, primary ciliary dyskinesia)
Intraluminal mass effects (foreign body aspiration,
tracheal/bronchial tumors, granulation tissue)
Dynamic airway collapse (tracheobronchomalacia)
Intrinsic narrowing of the airway (congenital or
acquired stenosis)
Extrinsic compression (vascular ring, mediastinal
lymph nodes or masses)

Differential Diagnosis
The Early Wheezer (<3 years old)

Wheeze associated
lower respiratory
tract infection

Early Onset Asthma

Febrile episodes
Personal atopy absent
Family history of
asthma/atopy absent
Variable response to
bronchodilators

Afebrile episodes
Personal atopy
present
Family history of
asthma/atopy present
Perdictable good
response to
bronchodilators

Broncholitis in children
Most common cause of wheezing in children aged 6 months to 3 years old
Diagnosis is mainly clinical
Commonly due to Respiratory Syncytial Virus (RSV)
Symptoms: Rhinorrhea, Pharyngitis, Cough, Low grade fever, Wheezing,
Tachypnea

Differential Diagnosis
Age

Common

Uncommon

Rare

<6
months

Broncholitis
Gastroesophageal
Reflux

Aspiration Pneumonia
Bronchopulmonary
Dysplasia
Congestive Heart Failure
Cystic Fibrosis

Asthma
Foreign Body
Aspiration

6 months
- 2 years

Broncholitis
Foreign Body Aspiration

Aspiration Pneumonia
Asthma
Bronchopulmonary
Dysplasia
Cystic Fibrosis
Gastroesophageal Reflux

Congestive Heart
Failure

2-5 years

Asthma
Foreign Body Aspiration

Cystic Fibrosis
Gastroesophageal Reflux
Viral Pneumonia

Aspiration
Pneumonia
Broncholitis
Congestive Heart
Failure

Jog your memory slide

What

is malacied here?
Trachea
Tracheomalacia
The hallmark?
expiratory wheeze,
hx of trauma due to?
Mechanical ventilation

What sign to you see?

Omega sign made by the epiglottis
collapse of?
supraglottic structures?
the arytenoid cartilages
and epiglottitis
When?

inspiratory stridor
Asthma is a disease of airway inflammation
Laryngomalacia

early

phase is the immediate response to an allergen, which causes

__?___to degranulate?
mast cells and basophils, which in turn precipitates?
bronchospasm as well as the release of proinflammatory cytokines and
chemokines
One of the most important risk factor for the development of asthma
is ?
Atopy

Practical
Management
of Asthma

Initial Assessment
Once

asthma has been diagnosed, the

degree of severity needs to be determined

Severity is best determined at the time of

diagnosis, before the initiation of therapy

Severity
1.
2.
3.
4.

can be divided into four categories

Intermittent
Mild Persistent
Moderate Persistent
Severe Persistent

*All individuals who have persistent asthma

require long-term control medication

Initial Assessment (continued)

Identify

and address precipitating factors (asthma

triggers)

Prick skin testing or blood testing (IgE) to detect

sensitization to common indoor allergens

The

most effective programs to reduce indoor allergens

are intensive, multifaceted interventions that address
more than one allergen

Common allergens
House

dust mite reduce indoor humidity, laundering

bedding in hot water, reducing dust catchers
Cockroach antigen pest management program
Screen

for comorbid conditions

Infection, obesity, depression in child or parent,

gastroesophageal reflux, allergies, obstructive sleep apnea

Medical Management
Two

types of medication are used to

treat asthma

1. Long-term control (prevention)

medications

Inhaled corticosteroids (ICSs) are the medication of

choice for all individuals suffering persistent
asthma

2. Quick relief medications

Reverse acute airflow obstruction

Children 0 to 4 Years

Children 0 to 4 Years

Children 0 to 4 Years

Children 5 to 11 Years

Children 5 to 11 Years

Children 5 to 11 Years

Youth 12 years of age and

Older

Youth 12 years of age and

Older

Youth 12 years of age and

Older

Corticosteroids
Inhaled

corticosteroids are first line therapy for chronic asthma

Examples
Mometasone
Fluticasone
Budesonide
Beclomethasone
Triamcinolone
Flunisolide

Mechanism:

inhibits the synthesis of virtually all cytokines and

inactivates NF-B (NF-B is the transcription factor that
induces the production of TNF- and other inflammatory
agents)
Toxicity
Oral

Candidiasis, Dysphonia (hoarseness), Bronchospasm, Reflex

Cough

use spacers (VHC) or post-inhalation mouth rinse to prevent

2-agonists
Drugs

Short acting -- used for breakthrough symptoms and during acute exacerbation
Albuterol (known internationally as salbutamol)
Levalbuterol
Others used much less commonly

Terbutaline
Metaproterenol (2, minor 1)
Isoproterenol (nonselective)

tachycardia
may lead to cardiac death

Long acting -- used for maintenance in

combination with inhaled corticosteroid (never without)

Salmeterol

Tremors, arrhythmia

Formoterol

Mechanism:

2 receptors are activated on bronchial smooth muscle to

achieve bronchodilation. Stimulation of adenylate cyclase leading to
closing of calcium channels and ultimately the relaxation of smooth
muscles

Methylxanthines
Theophylline

(rarely used)
Mechanism: inhibition of phosphodiesterase,
leading to decreased cAMP hydrolysis which causes
bronchodilation

metabolized by P-450
blocks actions of adenosine

Toxicity

seizures
narrow therapeutic index
nausea/vomiting
arrhythmia

Muscarinic antagonists
Drugs

Ipratropium
Tiotropium

Mechanism:

competitive inhibition of
muscarinic receptors prevents
bronchoconstriction
Also used for COPD

Cromolyn
Prophylaxis

only!
Ineffective during an acute asthma
attack
Mechanism: prevents release of
mediators from mast cells
Rare Toxicity

Antileukotrienes
Drugs

Zileuton
5-lipoxygenase

pathway inhibitor blocks

conversion of arachidonic acid to
leukotrienes

Zafirlukast, Montelukast
Blocks

leukotriene receptors
Particularly effective in aspirin-induced
asthma

Omalizumab

Clinical use

Severe uncontrolled asthma with elevated IgE

Mechanism: anti-IgE antibody (inhibits

action of IgE with inflammatory cells)

Symptoms and activity refractory to standard

therapies and oral glucocorticoids

Asthma can be caused by uncontrollably high

IgE response

Severe allergic reactions may occur

following infusion of omalizumab

Acute Exacerbation

Signs and symptoms of a severe exacerbation

Dyspnea at rest
Peak flow rate less than 40% of predicted or personal best
Accessory muscle use
Failure to respond to initial treatment

Initial management includes:

Brief assessment
Administration of a SABA to reverse airway obstruction

Add inhaled anticholinergic medications for moderate-tosevere exacerbations

Oxygen should be administered to most patients, particularly
those experiencing hypoxemia or moderate or severe
exacerbation
Systemic corticosteroids should be administered early in the
treatment of moderate or severe exacerbations and to any
patient who does not respond promptly to initial treatment

Question
Based on the NHLBI EPR 3 asthma guidelines, the most
preferred first step in therapy for moderate persistent
asthma would be which of the following?
A.
B.
C.

D.

Short-acting beta
agonists
Low-dose inhaled
corticosteroids
Low-dose inhaled
corticosteroids and longacting bronchodilator
High-dose inhaled
corticosteroids and longacting bronchodilator

 The

preferred treatment for moderate

persistent asthma is low-dose inhaled
corticosteroids and long-acting beta
agonists, leukotriene receptor
antagonist, or theophylline. Alternative
therapy would be medium-dose inhaled
corticosteroids.

Question:

12 year old female with a history of moderate

persistent asthma presents for routine follow-up. At the time of her
initial visit, you prescribed low-dose inhaled steroids and a
leukotriene modifier. She reports that since her initial visit, she has
had minimal daytime symptoms. She has required her rescue
inhaler only 2-3 times a week and awakes form sleep only about 3-4
times a month. She reports that, overall, she feels the medications
are working great. She denies significant exercise limitation. She has
had no exacerbations requiring oral steroids or acute intervention by
another physician. Based on the history provided by the patient, you
would classify her control as which of the following?

A.
B.
C.
D.

Well controlled
Not well controlled
Very poorly controlled
Unable to assess based on
this information

 The

patient meets 2 of the criteria for not

well controlled:
1)

Symptoms >2 times a week requiring rescue

inhaler
2) Nocturnal symptoms >2 times a month
The

patient also meets 1 criteria for well

controlled:
1)

Has had no oral steroids, ER visits, etc.

Therefore,

the patient is classified as not well

controlled because it is the the most severe
category in which her symptoms are
consistent

Question
All of the following are true regarding mechanisms
of action of inhaled glucocorticoid therapy except:
A.
B.
C.
D.

E.

Inhibition of cytokine
production
Inhibition of inflammatory
cell recruitment
Inhibition of mediator
release
Decreases microvascular
leak therefore decreases
edema formation
Down-regulation of betaadrenergic receptors

 Inhaled

glucocorticoids actually cause

up-regulation of beta-adrenergic
receptors. At one time, their use was
limited to patients with moderate-tosevere asthma, but now they are
recommended as first-line agents for
those with mild persistent asthma.
Clinically significant adverse reactions
are unlikely with appropriate pediatric
doses.

Jog your memory slide

Asthma

is a disease of airway inflammation

phase is the immediate response to an allergen, which causes __?___to
degranulate?
mast cells and basophils, which in turn precipitates?
bronchospasm as well as the release of proinflammatory cytokines and chemokines
One of the most important risk factor for the development of asthma is ?
Atopy
Rule of 2, converts asthma from intermittent to ?
Persistant
How do you treat differently?
early

Nurse

Any child with feeding abnormalities,

should undergo?
Barium Swallow test
What do you see here?

Vascular rings
Structural Abnormalities of the aortic arch
compressi ng trachea and esophagus
Stridor, Dysphagia

unable to pass a?
Nasogastric tube (NG)
Clinically?
Inspiratory stridor
cyanosis w/ feeding & resting
relieved by crying

Choanal atresia/Stenosis

nasal passage (choana)

Cystic
Fibrosis

Pathophysiology
Inherited

multisystem disorder in Caucasians,

disordered exocrine gland function

CFTR

gene is a cAMP activated chloride channel

on apical surface of epithelial cells in airways,
pancreatic ducts, biliary tree, intestines, vas
deferens and sweat glands.

Chloride

anions stay inside the cell--

Dehydration, viscid secretions and impairment
of mucociliary clearance.

Pathophysiology: Mutated
CFTR gene

Genetics/Epidemiology
Autosomal

Recessive- mutations of both alleles

of 250,000 bp gene on chromosome 7 called
cystic fibrosis transmembrane conductance
regulator (CFTR).
Most common defect Delta 508, deletion of 3
bps leading to absence of phenylalanine at
codon 508.
1/3500 newborns
Class I, II, III most severe defect in CFTR-
severe progressive pulmonary disease and
pancreatic insufficiency
Class IV, V- Milder

Clinical Manifestations
RESPIRATORY
Chronic productive
cough

GASTROINTESTINAL
Protein/fat
Malabsorption

OTHER SYSTEMIC
Diabetes Mellitus

Lower airways
Malnutrition/ FTT
bacterial colonization

Digital clubbing

Endobronchial
infection

Meconium Ileus

Hyponatremic
Dehydration

Exercise Intolerance

Distal Intestinal
Hypochloremic
obstruction syndrome Alkalosis

Hypoxemia

Obstructive Jaundice

Bronchiectesis

Focal Biliary Cirrhosis Zinc deficiency

Dermatitis

Pneumothorax

Rectal Prolapse

Hemoptysis

Recurrent Pancreatitis Nasal polyposis

Vitamin A, D, E, K
deficiences

Male infertility

Diagnosis
Test

any child with persistent cough, pneumonia, sinusitis or

unexplained poor weight gain/FTT.
Other diagnostic criteria include nasal polyps, rectal prolapse,
hypochloremic alkalosis, or known family history of the disease.
Diagnostic tests for CF include:
1-Newborn screening + when increased immunoreactive
trypsinogen (IRT)(pancreatic enzyme)
2-Confirmatory Sweat Chloride Test=Gold standard
Pilocarpine iontoelectrophoresis >60 meq/L -- +
3-DNA analysis (Definitive diagnosis with 2 CFTR mutations)
4-Nasal

potential difference test

(After chemical washing, patients with CF

show no change in measured electrical potential)

Diagnostic Criteria
BOTH

of the following criteria must be met to

diagnose CF
1- Clinical symptoms of CF in at least one
organ system AND
2- Evidence of CFTR dysfunction ( any of the
following)
Elevated sweat chloride >60 mmol/L on two
occasions
Presence of 2 disease causing mutations in
CFTR from each parental allele
Abnormal nasal potential difference.

LUNG DISEASE
Progressive,

obstructive lung disease with

thickening of airway mucus. Destroys lung
parenchyma, predominantly in conducting and
respiratory airways. Can lead to fibrotic
cavitations and diffuse cystic changes seen on
CXR.

LUNG DISEASE
On

PE: Coarse crackles, absent air movement,

tachypnea, air trapping and increased A/P chest
diameter.
Other findings include: Hypoxemia, exercise
intolerance, wt loss and decreased pulmonary function
Can assess FEV1, FEF 25-75 and lung volume
measurements
6-10 years pathogenic organism Staph Aureus
25-34 years Pseudomonas Aerginosa
Advanced CF: Burkholderia cepacia associated with
poor overall outcome

Complications
Pulmonary hemorrhage and spontaneous
pneumothorax.
Due to airway inflammation that erodes the bronchial
artery.
Massive hemoptysis of >500 ml in 24 hours will
require acute arterial embolization.

Pulmonary therapies
Check sputum/bronchoalveolar lavage for organisms
present
Keep in mind CF patients clear aminoglycosides more
rapidly and require increase in dosing.
Chronic P. aerginosa with inhaled tobramycin daily
every other month
3 times weekly azithromycin
Airway Clearance Therapy: percussive and postural
drainage
Older patients: oscillating chest vests
Hypertonic saline/recombinant human
deoxyribonuclease nebulized
Patients with respiratory failure are candidates for lung
transplantation

Postural Drainage

Upper Airway Disease

Opacification of maxillofacial sinuses on
radiography
Acute sinusitis, sinus tenderness, pressure
headaches, facial swelling
Antibiotic therapy for greater than 2 weeks,
depending on organism

NASAL POLYPS: Routine application of nasal

steroids, surgical intervention for nasal polyposis
Indication for sweat chloride testing in otherwise
healthy child.

Prognosis
Median predicted survival in 1985 was 25 years of age, by
2006 has risen to 37 years.
If diagnosed early by NBS can live into adulthood.
Early diagnosis, aggressive airway clearance therapies,
antibiotics and assessment of comorbidities is important.
Respiratory therapy for 60 minutes; 2-3 therapies per day!
Local CF centers have resources to help families with
financial need.
PCPs are the first ones to recognize early exacerbations,
give annual influenza vaccine.

Board Review: Clinical Vignette

A 2 year old male comes to your primary care office with the
complaint of chronic cough, which has been present since the
first few months of life. When he is very physically active he
sometimes wheezes. He has an uncle with asthma and his
parents have treated his wheezing with the uncles
bronchodilator inhaler without discernable improvement. He has
two older siblings who are healthy. His height is at the 30 th
percentile and his weight is below the 5 th percentile for his age.
His chest is slightly hyperinflated. Auscultation of the chest is
normal. While in the examination room he fills his diaper with
stool and the odor is foul smelling. On CXR: mild hyperinflation
is seen and bronchial thickening but is otherwise unremarkable.

Question
The sweat test result is 50 mEq/L and the laboratory
reports that the diagnostic value is >60mEq/l. Which of
the following in the most appropriate next response?
A.

B.
C.

D.
E.

measure pancreatic enzyme

concentration in a duodenal
aspirate
send blood for CFTR genotyping
reassure the family that the
sweat test is negative and the
child does not have CF
repeat the sweat chloride test
send the child to a research
center for the measurement of
nasal mucosal electrical
potential difference.

Answer
D.

Repeat the sweat chloride test: If the result

was 50 must repeat. Many patients with CF
may have a false negative sweat chloride test.

CTFR

genotyping is more expensive and time

consuming, but can be used as a primary
diagnostic tool.

Respiratory
Failure

Respiratory Failure
Acute respiratory failure is impairment in
oxygenation or ventilation where
PO2 < 60mmHg (acute hypoxemia)
PCO2 > 50 mmHg (acute hypercapnia)
pH < 7.35.

Respiratory Failure is basically an inability

of the respiratory system to meet the
metabolic needs of the tissues.

Incidence
Respiratory failure is inversely related to age in Peds
2/3rd of cases occur in 1st postnatal year
while seen in the neonatal period
Several developmental phenomenon are responsible
for the above stats

Airway is small with narrowest point in the subglottic

area. The cone shaped larynx is prone to obstruction
Thoracic cage is soft, with ribs positioned horizontally
which is a mechanical disadvantage for the chest
expansion
The diaphragm fatigues easily due to limited energy
stores in infants.
Immature nervous system often triggers
bradypnea/apnea

Causes
Failure

of oxygenation (Hypoxia)

Due to Imbalance of ventilation and

perfusion
(V/Q Mismatching)
OR

Due to impairment of oxygen diffusion at the

level of alveolar-capillary membrane

Failure

of Ventilation (Hypercapnia)

Decreased tidal volume (shallow breathing)

Decreased Respiratory Rate (bradypnea)

Classification of Hypoxia

Anoxic
Can be improved with increased inspired oxygen
But if its a result of shunt, increasing inspired
oxygen doesnt improve hypoxia

Anemic
Oxygen carrying capacity is impaired (Low
hemoglobin)
Or Insufficient functional hemoglobin
(Hemoglobinopathies)

Stagnant
When total blood flow is decreased (Heart
Failure)
Maldistribution of blood (Septic shock)

Cytochemical

Exogenous or endogenous factors which leads

to malfunctioned diffusion of oxygen at the level
of tissue (Cyanide)

Causes of
Respiratory Failure

Respiratory Failure
Pediatrics in Review 2009;30;470
Mara E. Nitu and Howard Eigen

Respiratory Failure
Pediatrics in Review
2009;30;470
Mara E. Nitu and
Howard Eigen

 Clinical

features: flushing, agitation and

tachycardia point to Acute hypercapnia

In

this case, lower airway obstruction led to poor

ventilation and respiratory failure.

RSV
1)
2)

can cause respiratory failure by two

mechanisms
Lower airway involvement (bronchiolitis as in this
case)
RSV caused central Apnea. (This is more frequent
in young infants than in older children)

Clinical Manisfestations
Case 1

A 4-month-old, previously healthy baby is seen in December for fever, a 4day history of nasal congestion, and progressive difficulty breathing.
Vital signs are: heart rate of 169 beats/min, respiratory rate of 56
beats/min, blood pressure of 126/56mmHg, and oxygen saturation on room
air of 92%. The infant is crying but can be consoled. Physical examination
reveals intercostal and subcostal retractions, tachypnea, bilateral
wheezing, and coarse breath sounds. Capillary refill is brisk and the
extremities are warm. A chest radiograph shows peribronchial cuffing and
slight hyperinflation suggestive of viral pneumonitis. A swab for respiratory
syncytial virus (RSV) is reported as positive. Supplemental oxygen is
initiated, viral bronchiolitis is diagnosed, and the infant is admitted for
monitoring. A few hours later, he becomes very agitated flushed, and
inconsolable. His heart rate is 189 beats/min, respiratory rate is 86
beats/min, and oxygen saturation is 92% on 3 L of oxygen administered by
nasal canula. His work of breathing is significantly increased, as
demonstrated by nasal flaring, grunting, head bobbing, and
significant retractions. The infant is transferred to the intensive care unit
for intubation and mechanical ventilation. Arterial blood gas before
intubation shows a pH of 7.16 and PCO2 of 70 mm Hg. He is intubated and
mechanically ventilated for 4 days.

Case 2

A 9-year-old child who has a history of Down syndrome, mitochondrial

myopathy, and tracheostomy is admitted because of a 3-week history
of decreased activity and increased somnolence. His respiratory rate
is 35 beats/min with very shallow effort. Arterial blood gas reveals:
pH, 7.33; PCO2, 62 mm Hg; PO2, 54 mm Hg; and HCO3, 28
mEq/L on room air (0.21 FiO2). Complete blood count reveals
polycythemia with a hemoglobin of 15 g/dL (150 g/L) and hematocrit
of 48% (0.48). The patient receives 100% oxygen, and
subsequent arterial blood gas determination documents pH,
7.23; PCO2, 80 mm Hg; PO2, 118 mm Hg; and HCO3, 32 mEq/L.
Chest radiograph reveals mild cardiomegaly and increased
pulmonary markings suggestive of chronic lung disease (Fig. 2).
Echocardiography shows mild pulmonary hypertension and right
ventricular hypertrophy. The patient is placed on home mechanical
ventilation to treat chronic respiratory failure.

Whats going on?!

Patients with underlying myopathy lack the ability to

increase the work of breathing (first sign of respiratory
failure)
Thus, such patients present with tachypnea and very shallow
breathing without any rib retractions

The first gas pattern: Typical of Chronic respiratory

failure. Mild Respiratory acidosis (increased PCO2)
with metabolic compensation (increased bicarb)

Patients with chronic respiratory failure, if given 100%

oxygen, can lead to higher retention of carbon
dioxide [These patients respiratory center is primarily
stimulated by hypoxic drive and with 100% oxygenation, this
drive gets blunted]

Polycythemia, Pulmonary hypertension and cor

pulmonale seen in this patient represents rare

Chest
Radiograph
showing
Cardiomegaly and
increased
pulmonary
markings [Case 2]

Respiratory Failure
Pediatrics in Review
2009;30;470
Mara E. Nitu and
Howard Eigen

History and Physical

Examination

Initial Assessment: Urgent medical Intervention

Helpful indicators: Vital signs, work of breathing

and level of consciousness

Patient with rapid respirations,

significant retractions, head
bobbing, nasal flaring, grunting
As patient becomes fatigued,
there are more shallow
respirations, leading to lack of
responsiveness and
hypoxemia despite a high
FiO2
Patient presenting with severe
hemodynamic compromise,
mottled extremeties, and very
low blood pressure

Aggressive and
urgent
respiratory
support

Emergent airway control

and ventilatory support.

Emergent airway control,

breathing and circulatory
support

 If

emergent Intervention is not necessary, a

comprehensive history can be obtained to
determine the probable causes of respiratory
failure:

Previous fever and ill contacts

Possible foreign body aspiration
Previous chronic lung disease (Cystic Fibrosis,
Asthma, Prematurity)
Causes of central ventilation (drug ingestion, head
trauma and seizures)
Physical Signs:

Vital signs (Respiration rate, Heart rate, Blood Pressure)

Pulse Oximetry

But it is unreliable for patients with decreased tissue

perfusion (shock, hypovolemia or hypothermia)

Clinical Findings: Increased work of breathing Nasal

Flaring, substernal retractions, head bobbing, respiratory
pauses, grunting and thoracoabdominal asynchrony,
shape of the thoracic cage and spine should be noted

Asymmetric chest movements, tracheal deviation point at

plueral effusion or pneumothorax

Auscultation: Presence of wheezing or crackles, stridor,

decreased breath sounds, heart sounds, palpation of
brachial/femoral pulses

Asymmetric wheezing Foreign body Aspiration or

mass obstructing the airway
Crackles Alveolar process such as Pneumonia
Abnormal heart sounds Congenital or acquired heart
disease
Palpation of brachial/femoral pulses Help rule out
aortic coarctation

Clinical Findings
Intercostal and
Substernal retractions

Respiratory Failure
Pediatrics in Review 2009;30;470
Mara E. Nitu and Howard Eigen

Head
Bobbing

Respiratory Failure
Pediatrics in Review 2009;30;470
Mara E. Nitu and Howard Eigen

Thoracoabdominal asynchrony
Respiratory Failure
Pediatrics in Review 2009;30;470
Mara E. Nitu and Howard Eigen

 Assessment

of Muscle Strength and Gait may

point to underlying reason of respiratory failure

Decreased muscle strength Duchene muscular

dystrophy or some mitochondrial diseases
Delayed or loss of motor milestones first clues
to severe myopathy
Lack of attaining head control Spinal muscular
atrophy
Acute ascending paralysis . Guillain-Barre
Syndrome
Acute generalized muscle weakness Botulism

Assessment of mental status using Glasgow Coma

Scale (GCS)

Decreased GCS Impaired Neurologic function

GCS < 8 Airway control by intubation and
mechanical ventilation

Laboratory and Radiographic Evaluation

Arterial blood gas Assesses the extent of hypoxemia or

hypercapnia

Blood gas analysis should be correlated with the clinical picture

A normal PCO2 with high work of breathing and severe
tachypnea reflects respiratory failure
Normal PCO2 with severe metabolic acidosis and tachypnea
impending respiratory failure

Respiratory Failure
Pediatrics in Review 2009;30;470
Mara E. Nitu and Howard Eigen

Complete blood count

Chest Radiograph

Anemia can be associated with chronic illness

and polycythemia and obstructive sleep
apnea

Pneumonia, Pulmonary edema, Pneumothorax, or

pleural effusion

Pulmonary function testing

Characterize the respiratory disease, its severity,
course of illness and response to therapy

Management
Close

monitoring and supplemental oxygen to full

mechanical ventilatory support.
Rapid initial assessment Emergency intervention,
preparation for intubation and mechanical
ventilation

Bag-mask ventilation with 100% oxygen (For proper

ventilation)
Intubation
Neuromuscular

blockade can be used as well for difficult

airway management.
Laryngeal mask airway (LMA) is another option if intubation
is difficult (LMA has its limitation since it doesnt protect
against aspiration in the patient who hasnt been fasting)

 If

no emergency intervention needed:

Mild cases Supplemental oxygen delivered via
nasal canula
O2 requirements high nonrebreather mask can be
used, that delivers high flow O2 at 10-15L/min
NonInvasive Mechanical Ventilation

If

CPAP or BiPAP
[The risk of developing sores on the face limits prolonged use
of noninvasive mechanical ventilation for 24 hours a day]
BiPAP can be used at home for chronic respiratory failure to
postpone the need for tracheostomy or home mechanical
ventilation or who choose not to use other interventions.

Conventional treatment of acute respiratory

failure

Positive pressure ventilation + Supplemental O2

Ventilation with elevated O2 concentrations and
airway pressure has been shown to worsen lung
injury [Ventilator induced lung injury (VILI)].
Thus goal is to minimize lung injury while
providing effective ventilation and
oxygenation
An FiO2 > 0.6 used for longer than 6 hours is
believed o add oxidative stress to the ventilated
lungs and contribute to VILI
At present, use of lowest possible pressures
and volumes to maintain acceptable
ventilation and oxygenation is recommended.

The instillation of surfactant and inhaled nitric oxide

(iNO) can supplement mechanical ventilation in carefully
select patients.

Intratracheal instillation of surfactant in preterm infants has

significantly improved the outcome of RDS

iNOS is an adjunctive therapy for patients who have

documented or suspected pulmonary hypertension and
significant oxygenation failure.

If adequate gas exchange cant be achieved with conventional

mechanical ventilation High frequency ventilation is a
good therapeutic option.

When all options have failed to provide adequate gas exchange

and hemodynamic support Extracorporeal membrane
oxygenation (ECMO)

Weaning from mechanical ventilation is achieved

gradually as the underlying pathologic process resolves.

Summary
There

are many conditions that can lead to

respiratory system to meet the metabolic
needs of the tissues
Prompt interventions and close monitoring in
the critical care setting is important to avoid
cardiorespiratory arrest secondary to
unrecognized respiratory failure.
Clinical interventions range from non invansive
methods to intubation and mechanical
ventilation to ECMO as the last resort

Jog your memory slide

If you get a child with

respiratory distress and
ABG reveals an oxygen
tension of 45 mm Hg and
a carbon dioxide tension
of 60 mm Hg. What
should you do?
Intubate- insert
Endotracheal tube

Newborn screening + when?

increased immunoreactive
trypsinogen (IRT)(pancreatic enzyme)
Confirmatory Sweat Chloride Test is?
=Gold standard
Pilocarpine iontoelectrophoresis >60
meq/L -- +
DNA analysis (Definitive diagnosis with?
2 CFTR mutations
Most common defect is?

defect Delta 508, deletion of 3 bps

leading to
absence of phenylalanine at codon
508.

SIDS
Sudden infant death
syndrome

Definition
SIDS

sudden unexplained death before

1 year of age in an otherwise healthy
infant. The cause of death is
unexplained despite a thorough
investigation involving:

Complete autopsy
Death scene investigation
Review of clinical history

Epidemiology
3rd

leading cause of death in infants in ages 1

mos 1 year.
2,300 infants die of SIDS every year in the U.S.
There has been a steady decline in death from
SIDS with the implementation of Back to Sleep
campaign that started in 1994.
SIDS rate has been constant since 2001.
Many deaths that were previously classified as
SIDS are now being classified as other causes
of death.

Risk Factors
Male infants (occurs 3:2 compared to females)
Prone and side sleeping position
Maternal smoking during pregnancy
Exposure to tobacco smoke
Overheating
Soft bedding
Young maternal age
Prematurity or low birth weight
African American/American Indian

***Risk Reduction****
Strategies

to decrease risk factors

Back to sleep for every sleep

Use firm sleep surface
Keep soft objects and loose bedding out of crib
Avoid tobacco smoke exposure during
pregnancy
Room sharing without bed sharing is
recommended
Avoid overheating

Pathophysiology
Failure of arousal mechanisms plays a big role in
the pathway to death
Dysfunction of arousal and cardiorespiratory
responses may be due to serotonin receptor
abnormalities
Other causes may be polymorphisms in sodium
channel gene that relates to prolonged QT
syndrome
Certain genes seem to predispose infants to SIDS
especially when they are exposed to smoking or
prone position

Sleep position
Evidence

suggests that prone sleeping

results in altered autonomic control of
the infant cardiovascular system during
sleep

Seen at 2-3 mos of age

Get decreased cerebral oxygenation

Some

parents are reluctant to try supine

position because they fear infant will
choke or aspirate

Bed Sharing
AAP

recommends infants share a room with

the parents, but not a bed
Decreases risk of SIDS by 50%
Adult beds are not designed for infant safety

May lead to accidental suffocation

Entrapment
Asphyxia

Highest

incidence of SIDS is when bed sharing

occurs during first 3 months of life and if they
are born prematurely

Bed Sharing cont.

Hazardous

bed-sharing situations:

Infant <2 months

Parents are smokers
Infants placed on water beds or sofas
(soft surfaces)
Pillows and blankets present
Person sharing a bed is not a parent
Person sharing a bed is intoxicated

Reasons for bed sharing

Space/financial reasons
Facilitates breastfeeding and bonding
Environmental dangers:

Vermin
Stray gunfire
Random kidnappings

Parents believe parental vigilance at all times is best

Recommendation is to room share to maintain

bonding and vigilance

Protective effects

Breastfeeding

Studies have shown that SIDS risk halves when baby is

breastfed
The risk decreases even more when the infant is
exclusively breastfed
Breastfeeding decreases infectious diseases which is
associated with SIDS
It is also easier to arouse infants from sleep that are
breastfed than those that are bottle fed

Pacifiers

AAP recommends pacifier use to decrease incidence of

SIDS

Protective effects cont.

Crib and Bedding accessories:

Current recommendation is to place infant on firm surface

Avoid bedding, pillows, blankets and use infant sleep
clothing
Avoid bumper pads that may cause suffocation,
entrapment, and strangulation
Parents

normally use to them to avoid injuries

Possibly for aesthetic reasons

Vaccinations

Studies have shown that immunization decreases risk of

SIDS

Health Education
Education about sleep position should be detailed and
include concerns of aspiration, choking, and infant
comfort
Media messages that target child-bearing woman often
depict infants in unsafe sleep positions and sleep
environments (soft-bedding)
Important to be consistent in message
Many parents question Back to Sleep campaign as SIDS
is said to have unknown cause so they dont believe
any changes in sleeping behavior could prevent death
of infants
Other simply believe it is Gods will

Question
Abnormalities in the receptor for which of the following
neuropeptides have been identified in the brainstem of
some infants with sudden infant death syndrome:
A.
B.
C.
D.
E.

Acetylcholine
Epinephrine
Galanin
Serotonin
Vasoactive intestinal
peptide

Question:

A mother of a 1-month-old infant comes in for a well-child visit. As

you review the infants immunization schedule, she tells you that one of her friends
children died of SIDS 48 hours after the infant received her 4-month diphtheria,
pertussis, and tetanus vaccine. Of the following, which piece of information most
accurately reflects current knowledge about immunizations and SIDS?

A.
B.

C.

D.

E.

Infants who receive immunizations have

a lower risk of SIDS
Infants who receive immunizations have
the same risk of SIDS as infants who are
not immunized
Infants who receive immunizations may
have slightly higher risk of SIDS, but the
benefit of the immunization far
outweighs the risk
Only the 2-month diphtheria, pertussis,
and tetanus vaccine has been
associated with slightly increased risk of
SIDS.
Infants at risk of SIDS should have their
Haemophilus influenza vaccine series
postponed until 12 months

Question
The American Academy of Pediatrics Task Force on SIDS has recommended
against parents sleeping in the same bed as their infants. According to the
article, which of the following factors makes bed sharing especially hazardous?

A.
B.
C.
D.
E.

Absence of blankets
Firm bed
Infants age <3 months
Maternal age <20 years
Only 1 person sharing
the bed with the infant

Question
An intervention recommended by authors to
reduce the risk of infant SIDS is:

A.
B.
C.
D.
E.

Avoid the use of pacifiers

before bedtime
Home cardiorespiratory
monitoring
Positioning the infant on
the side
Sleeping in the same room
as the infant
Swaddling the infant in a
soft blanket

Jog your memory slide

Treatment of Asthma?
B2 agonist/bronchdilators
Short acting for breakthrough symptoms
Ex. Albuterol (AKA internationally as salbutamol) Levalbuterol
Long acting -- used for maintenance in combination with inhaled
corticosteroid (never without)
Ex . Salmeterol
Mechanism?
2 receptors are activated on bronchial smooth muscle bronchodilation.
Stimulation of adenylate cyclase closing of calcium channels
relaxation of smooth muscles

Inhaled corticosteroids are first line therapy for?

Persistant asthma
Examples?
Mometasone
Fluticasone
Budesonide
Mechanism: inhibits the synthesis of cytokines
and inactivates NF-B that induces the
production of TNF- and other inflammatory
agents)

Newborn screening + when?

increased immunoreactive trypsinogen (IRT)(pancre
enzyme)
Confirmatory Sweat Chloride Test is?
=Gold standard
Pilocarpine iontoelectrophoresis >60 meq/L -- +
DNA analysis (Definitive diagnosis with?
2 CFTR mutations
Most common defect is?
defect Delta 508, deletion of 3 bps leading to
absence of phenylalanine at codon 508.

References

Hill, Vanessa L., and Pamela R. Wood. "Asthma Epidemiology,

Pathophysiology, and Initial Evaluation." Pediatrics in Review
30.9 (2014). Web. 4 Sept. 2014.
<http://pedsinreview.aappublications.org/content/30/9/331>.
Marino, Bradley S., and Katie S. Fine. "Pulmonology."
Blueprints Pediatrics. 5th ed. Lippincott Williams & Wilkins,
2009. 299-309. Print.
"Pathophysiology of Asthma." Wikipedia. 5 May 2014. Web. 6
Sept. 2014.
<http://en.wikipedia.org/wiki/Pathophysiology_of_asthma>.

References
Hill,

Vanessa L., and Pamela R. Wood. Practical

Management of Asthma" Pediatrics in Review 30.9
(2014). Web. 4 Sept. 2014.
<http://pedsinreview.aappublications.org/content/30/1
0/375>.
Marino, Bradley S., and Katie S. Fine. "Pulmonology."
Blueprints Pediatrics. 5th ed. Lippincott Williams &
Wilkins, 2009. 299-309. Print.
"Pathophysiology of Asthma." Wikipedia. 5 May 2014.
Web. 6 Sept. 2014.
<http://en.wikipedia.org/wiki/Pathophysiology_of_asth
ma>.

References
1.

2.
3.

4.

Hauck FR, Thompson JM, Tanabe KO, Moon RY,

Vennemann MM. Breastfeeding and reduced risk of
sudden infant death syndrome: a meta-analysis.
Pediatrics. 2011; 128(1): 103-110
Moon RY, Fu LY. Sudden infant death syndrome. Peiatr
Rev. 2007; 28(6): 209-214
Moon RY, Yang DC, Tanabe KO, Young HA, Hauck FR.
Pacifier use and SIDS: evidence for a consistently
reduced risk. Matern Child Health J. 2012;16(3):609-614
Tappin D, Ecob R Brooke H. Bedsharing, roomsharing,
and sudden infant death syndrome in Scotland: a casecontrol study. J Pediatr. 2005; 147(1): 320

References
1.

Montgomery GS, Howenstine M. Cystic

Fibrosis. Pediatrics in Review 2009; 30;302
2. Katkin, JP. Cystic Fibrosis: Genetics and
Pathogenesis. UpToDate. 2014.
3. Katkin, JP. Cystic Fibrosis: Clinical
Manifestations and diagnosis. UpToDate. 2014.
4. Marino, Bradley. Blueprints: Pediatrics 6th
edition 2013.

Seong J. Kim
MS IV
St. Georges University = croup and stridor
Adnan Ismail, MS IV

Aamer javed, ms4

Sgu
8/1/2014

Snehali Patel, MSIV

St. Georges University
June 20th 2014

Adnan Ismail, MSIV

Anousheh Afjei