PEDIATRIC DISORDERS | Prof.
Tere Vanguardia
RESPIRATORY SYSTEM
DISORDERS OF THE LOWER RESPIRATORY SYSTEM
I. Influenza
Influenza involves inflammation and infection of the major airways.
It is caused by the orthomyxovirus influenza type A, B, or C.
Signs and Symptoms
Cough
Fever
Fatigue
aching pains
a sore throat
gastrointestinal symptoms such as vomiting or diarrhea.
Management
II. Bronchitis
Bronchitis, or inflammation of the major bronchi and trachea
one of the more common illnesses affecting preschool and school-age children.
Signs and Symptoms
Fever
Dry hacking Cough
Nasal congestion.
Rhonchi
Coarse crackles (the sound of rales) can be heard
Diffuse alveolar hyperinflation and some markings at the hilus of the lung upon XRay
Management
III. Bronchiolitis
Bronchiolitis is inflammation of the fine bronchioles and small bronchi. It is the most
common lower respiratory illness in children younger than 2 years, peaking in
incidence at 6 months of age.
The infection occurs most often in the winter and spring.
Many children who develop asthma later in life have numerous instances of
bronchiolitis during their first year of life.
Viruses, such as adenovirus, parainfluenza virus, and RSV, in particular, appear
to be the pathogens most responsible for this illness
Assessment
Management
IV. Asthma
Asthma, an immediate hypersensitivity (type I) response, is the most common
chronic illness in children, accounting for a large number of days of absenteeism
from school
and many hospital admissions each year. It tends to occur initially before 5 years of
age.
Signs and Symptoms
Diagnostic Procedure
Pulmonary Function Studies
Children are assigned “zones” to rate their expiratory
compliance:
• Green zone (80% to 100% of their personal best) means no asthma
symptoms are
present, and they should take their routine
medications.
• Yellow zone (50% to 80% of personal best) signals caution. An episode of
asthma
may be
beginning.
• Red zone (below 50% of personal best) indicates an asthma episode is
beginning.
Children should immediately take their prescribed medication such as an
inhaled
beta-2–agonist, then repeat the peak flow assessment. If the second reading is
not in the green zone, their parents should alert their primary care provider of the
impending asthma attack.
Management
anti-inflammatory corticosteroid
bronchodilator
mast cell stabilizer (cromolyn sodium
leukotriene receptor antagonists (montelukast)
Complication
Status Asthmaticus
V. Bronchopulmonary Dysplasia
Bronchopulmonary dysplasia (BPD) is chronic pulmonary involvement that occurs in
10% to 40% of infants who are treated for acute respiratory distress in the first
days of life
Most often occurs in infants who received mechanical ventilation for respiratory
distress syndrome at birth.
It occurs most often in infants born weighting less than 1000 g.
Signs and
Symptoms
Management
MMC
VII. Cystic Fibrosis
Children with cystic fibrosis (CF) have a generalized dysfunction of the exocrine glands
Autosomal recessive
Signs and Symptoms
Pancreas Involvement
Absence of pancreatic enzymes
Steatorrhea
Vitamin ADEK deficiency
Malnutrition
protuberant abdomen
meconium ileus
Rectal prolapse
Lung Involvement
Clubbing of fingers
Chronic Productive Cough
Enlarged anterior-posterior diameter of the chest
Sweat Gland Involvement
Salty taste
Increase chloride level in sweat
Diagnostic Procedure
Management
Therapy for children with CF consists of measures to reduce the involvement of
the pancreas, lungs, and sweat glands. Because so many organs are involved,
care works best if it is a collaborative process
Humidified Oxygen
Aerosol Therapy. Three or four times a day, children may be given aerosol
therapy by means of a nebulizer to provide antibiotics or bronchodilators.
Antibiotics are specifically determined by culture.
Mucolytic, such as acetylcysteine (Mucomyst), can be added to the mist to aid in
diluting and liquefying secretions.
Chest Physiotherapy. To aid drainage of secretions, children need chest physiotherapy
frequently, approximately three or four times a day.
Activity. Children with CF need to maintain their usual activities as much as possible. When
in bed, they need frequent position changes so that, at various times of the day, all
lobes of their lungs will be encouraged to drain by being in a superior position.
Respiratory Hygiene. The sputum that a child coughs up may have a disagreeable taste or
odor. Offer frequent mouth care, toothbrushing, and a good-tasting mouthwash to make the
child’s mouth feel fresh.
CARDIOVASCULAR SYSTEM
DISORDERS OF THE CARDIOVASCULAR SYSTEM
I. Congenital Heart Defects
Types
A. Increased pulmonary blood flow
B. Obstruction to blood flow leaving the heart
C. Mixed blood flow (oxygenated and deoxygenated blood mixing in the heart or
great vessels)
D. Decreased pulmonary blood flow
A. Disorders With Increased Pulmonary Blood Flow
1. Ventricular septal defect (VSD)
2. Atrial septal defect (ASD)
3. Atrioventricular canal (AVC) defect
4. Patent ductus arteriosus (PDA).
1. Ventricular Septal Defect
VSD, the most
common type of
congenital cardiac
disorder seen,
accounts for about
30% of all instances of
congenital heart
disease, or about 2 in
every 1000 live births
With this defect, an
opening is present in
the septum between
the two ventricles.
Assessment
Diagnosis
Therapeutic Management
Complications
2. Atrial Septal Defect
An ASD is an abnormal
communication between the
two atria, allowing blood to
shift from the left to the right
atrium (an acyanotic
defect).
It is more common in girls
than boys
Assessment
Diagnosis
Therapeutic Management
Complications
3. Atrioventricular Canal Defect
AVC, also called an
endocardial cushion defect, results
from incomplete fusion of the
endocardial cushion, which is the
septum of the heart at the junction
of the atria and the ventricles
Although rare in the general
population, as many as 50% of
children with trisomy 21 (Down
syndrome) who have heart
disease have this type of
congenital cardiac defect
Assess
ment
The defect leads to the
same symptoms as other ASDs
(right ventricular hypertrophy,
increased pulmonary blood
flow, and fixed S2 splitting).
Diagnosis
An ECG often will reveal first degree heart block as impulse conduction is halted
before the AV node.
Echocardiography will confirm the diagnosis.
Management
Pulmonary artery banding may be done palliatively in selected infants. This
increases pressure in the pulmonary artery and right side of the heart,
reducing the amount of shunting.
Surgery, however, is always necessary for a final repair because these defects
are too large to close spontaneously. Because surgery may involve a valve
repair as well as a septal repair, mitral and tricuspid insufficiency from poor
valve function may occur at a later date.
4. Patent Ductus Arteriosus
The ductus arteriosus is an accessory fetal structure that connects the
pulmonary artery to the aorta. If it fails to close at birth (closure begins with the
first breath, and is usually complete between 7 to 14 days of age, although full
closure may not occur until 3 months of age), blood will shunt from
PDAs are twice as common in girls as boys and occur at a higher incidence at
higher altitudes.
In preterm infants,the incidence may be as high as 20% to 60% and
accounts for about 10% of all heart disease.
Assessment
Diagnosis
Therapeutic Management
B. Disorders with Obstruction to blood flow leaving the heart
1. Pulmonary stenosis
2. Aortic stenosis
3. Coarctation of the aorta.
1. Pulmonary Stenosis
Pulmonary stenosis is narrowing of the pulmonary valve or the pulmonary
artery just distal to the valve
It accounts for about 10% of congenital heart anomalies
Assessment
asymptomatic
signs of mild (right-sided)
heart failure
severe: cyanosis
thrill may be present in the
upper left sternal area or at the
suprasternal notch
Split S2
Diagnosis
ECG or echocardiography will
reveal right ventricular
hypertrophy
Cardiac catheterization is rarely
necessary for diagnosis but is
used for interventional
enlargement of the stenosed
valve.
Therapeutic Management
Balloon angioplasty by way of cardiac catheterization
2. Aortic Stenosis
Stenosis, or stricture, of the aortic
valve prevents blood from passing freely
from the left ventricle of the heart into the
aorta.
Aortic stenosis accounts for about
7% of congenital cardiac abnormalities
Assess
ment
asymptomatic
,
typical
murmur
thrill may be present, particularly at
the suprasternal notch
If severe, decreased cardiac output
evidenced by faint pulses, hypotension,
tachycardia, and inability to suck for long
periods may be present
When the child is active, chest pain
similar to angina
Diagnosis
ECG or echocardiography will reveal left ventricular hypertrophy
Cardiac catheterization is rarely necessary unless interventional therapy by this route is
planned.
Therapeutic Management
beta-blocker or a calcium channel blocker
valvuloplasty
artificial valve replacement for correction
3. Coarctation of the Aorta
Coarctation of the aorta, a narrowing of the lumen of the aorta due to a constricting band
This accounts for about 6% of instances of congenital heart disease
It occurs more frequently in boys than in girls and is the leading cause of congestive
heart failure in the first few months of life.
There are two locations in which this commonly occurs. In the first, termed “preductal,”
the constriction occurs between the subclavian artery and the ductus arteriosus. In the
second, postductal, the constriction is distal to the ductus arteriosus.
Assessment
headache and vertigo.
exceptional irritability (below 3 years
old)
Epistaxis (nosebleed)
cerebrovascular accident
absence of palpable femoral pulses
leg pain on exertion because of the
diminished blood supply to their lower
extremities
blood pressure in the arms will be at
least 20 mm Hg higher than in the legs
Diagnosis
Echocardiography
ECG
MRI
X-ray examination
Therapeutic Management
Interventional angiography (a balloon catheter)
Surgery
C. Disorders With Mixed Blood Flow
1. Transposition of the great arteries
2. Total anomalous pulmonary venous return
3. Truncus arteriosus
4. Hypoplastic left heart syndrome.
1. Transposition of the Great Arteries
In transposition of the great arteries, the
aorta arises from the right ventricle instead
of the left, and the pulmonary artery arises
from the left ventricle instead of the right.
This severe a defect is incompatible with life.
In most instances, atrial and ventricular
septal defects occur in connection with this
transposition, making the entire heart one
mixed circulatory system.
It tends to occur in large newborns (9 to 10
lb) and occurs more often in boys than in
girls.
This disorder accounts for about 5% of
congenital heart anomalies
Assessment
cyanotic from birth
Diagnosis
Echocardiography generally reveals an enlarged heart
Cardiac catheterization will reveal the low oxygen saturation resulting from the mixing of
blood in the heart chambers.
Therapeutic Management
Within a few days, ideally 24 hours after birth
PGE1
A balloon atrial septal pull-through operation
2. Total Anomalous Pulmonary Venous
Return
In this disorder, the pulmonary veins return to
the right atrium or the superior vena cava
instead of to the left atrium as they normally
would.
This disorder accounts for only 2% of all
congenital heart disorders.
Assessment
An absent spleen is often associated with the disorder.
mildly cyanotic
tire easily
Therapeutic Management
Surgery: involves reimplanting the
pulmonary veins into the left atrium.
3. Truncus Arteriosus
In truncus arteriosus, a rare defect
(approximately 1% of initial cardiac lesions),
one major artery or “trunk” arises from the left
and right ventricles in place of separate aorta
and pulmonary artery vessels
Assessment
There is usually an accompanying VSD
cyanotic
typical VSD murmur
Therapeutic Management
Surgery: restructuring the common trunk to
create separate vessels.
4. Hypoplastic Left Heart Syndrome
D. Disorders With Decreased Pulmonary Blood Flow
1. Tricuspid atresia
2. Tetralogy of Fallot.
1. Tricuspid Atresia
Tricuspid atresia is an extremely serious disorder because the tricuspid valve is
completely closed, allowing no blood to
flow from the right atrium to the right
ventricle
Assessment
extreme cyanosis
tachycardia
dyspnea
Therapeutic Management
2. Tetralogy of Fallot
Tetralogy of Fallot, one of the first
types of congenital heart disease
described
Occurs in about 10% of children with
congenital cardiac disease
It is called a tetralogy because four
anomalies are present:
1. P - pulmonary stenosis
2. O - dextroposition (overriding) of
the aorta
3. V - VSD (usually large)
4. R - right ventricular hypertrophy
Assessment
Diagnosis
History and physical symptoms
Echocardiography
ECG
Cardiac catheterization
Laboratory findings reveal polycythemia, increased hemoglobin, hematocrit, and total
red blood cell count as well as reduced oxygen saturation.
Therapeutic Management
II. ACQUIRED HEART DISEASE
HEMATOLOGIC DISORDERS
GASTROINTESTINAL SYSTEM
DISORDERS OF THE GASTROINTESTINAL SYSTEM
I. CLEFT LIP AND CLEFT PALATE
Assessment
Cleft lip may be detected by a sonogram while an infant is in utero.
Cleft palate can be determined by depressing the newborn’s tongue with a tongue blade.
This reveals the total palate and the extent of any cleft present.
Therapeutic Management
II. TRACHEOESOPHAGEAL FISTULA AND ATRESIA
Between weeks 4 and 8 of intrauterine life, the laryngotracheal groove develops into the
larynx, trachea, and beginning lung tissue. The esophageal lumen forms parallel to this.
Esophageal atresia is obstruction of the esophagus. Often a fistula (opening) occurs
between the closed esophagus and the trachea.
The five usual types of esophageal atresia that occur are:
1. The esophagus ends in a blind pouch; there is a tracheoesophageal fistula between the distal
part of the esophagus and the trachea
2. The esophagus ends in a blind pouch; there is no connection to the trachea
3. A fistula is present between an otherwise normal esophagus and trachea
4. The esophagus ends in a blind pouch. A fistula connects the blind pouch of the proximal
esophagus to the trachea
5. There is a blind end portion of the esophagus. Fistulas are present between both widely
spaced segments of the esophagus and the trachea
Assessment
Therapeutic Management
Emergency surgery
Antibiotics may be prescribed to help prevent infection.
A gastrostomy may be performed (under local anesthesia) and the tube allowed to drain
by gravity to keep the stomach empty of secretions and prevent reflux into the lungs.
It may be necessary to complete the surgery in different stages and to use a portion of
the colon to complete the anastomosis if the esophageal segments are far apart from
each other.
Observe infants closely at postoperative days 7 to 10, when sutures dissolve, because
leaks occurring at anastomosis sites can occur at this time. If this occurs, fluid and air
leak out into the chest cavity, and pneumothorax (collapse of the lung) can occur.
III. GASTROESOPHAGEAL REFLUX DISEASE
Gastroesophageal Reflux in Infants
Gastroesophageal reflux in infants occurs from a neuromuscular disturbance in which
the gastroesophageal (cardiac) sphincter and the lower portion of the esophagus spasm
and allow easy regurgitation of gastric contents into the esophagus.
It usually starts within 1 week after birth and may be associated with a hiatal hernia (see
later discussion).
Children with cerebral palsy or other neurologic involvement are at particular risk. The
regurgitation occurs almost immediately after feeding or when the infant is laid down
after a feeding.
Assessment
The diagnosis is suggested by the history.
Vomiting appears effortless and is not projectile; it begins much earlier in life than the
vomiting associated with pyloric stenosis.
The child may be irritable and may experience periods of apnea.
Inserting a probe or catheter through the nose into the distal esophagus and determining
the pH from secretions can show whether gastric secretions are entering the esophagus
(if the pH is less than 7.0, then acid is present).
Esophageal manometry is used to measure the strength of the esophageal sphincter.
Fiberoptic endoscopy or esophagography (barium swallow) will further show the involved
sphincter and the reflux of stomach contents into the esophagus, especially if the infant’s
head is tilted downward.
Therapeutic Management.
IV. PYLORIC STENOSIS
With this condition, at 4 to 6
weeks of age, infants begin to
vomit almost immediately after
each feeding. The vomiting grows
increasingly forceful until it is
projectile, possibly projecting as
much as 3 to 4 feet. The incidence
is high, approximately 1:150 in
males and 1:750 in females. It
tends to occur most frequently in
first-born white male infants.
Assessment
The diagnosis of pyloric stenosis is made primarily from the history. Whenever parents
say that their baby is vomiting or spitting up, be certain to get a full description:
What is the duration?
What is the intensity?
What is the frequency?
What is the description of the vomitus?
Is the infant ill in any other way?
V. INTUSSUSCEPTION
Intussusception, the invagination of
one portion of the intestine into
another, usually occurs in the
second half of the first year of life
In infants younger than 1 year,
intussusception generally occurs for
idiopathic reasons. In infants older
than 1 year, a “lead point” on the
intestine likely cues the invagination
Ileoileal
Ileocecal
VI. CELIAC DISEASE
Also known as Malabsorption Syndrome; Gluten-Induced Enteropathy
The basic problem in celiac disease is a sensitivity or abnormal immunologic response
to protein, particularly the gluten factor of protein found in grains—wheat, rye, oats, and
barley.
The illness occurs most frequently in children of a northern European background. It is
apparently a dominantly inherited illness although children have different degrees of
involvement.
There is also an increased incidence in children of type 1 diabetes mellitus, IgA
deficiency, and Down syndrome, suggesting an associated immune response etiology
Assessment
VII. HIRSCHSPRUNG’S DISEASE (AGANGLIONIC MEGACOLON)
Hirschsprung’s disease, or
aganglionic megacolon, is absence of
ganglionic innervation to the muscle of a
section of the bowel—in most instances, the
lower portion of the sigmoid colon just above
the anus
Assessment
GENITOURINARY DISORDERS
NEUROMUSCULAR DISORDERS
