Professional Documents
Culture Documents
Tooley
Intensive Care Nursery
Eighth Edition
July, 2003
This manual, now in its eighth edition, is designed for use by the pediatric residents,
interns and medical students who work in the William H. Tooley Intensive Care Nursery
at UCSF Medical Center. Therefore, the recommendations in this manual are specific for
the practices in that unit. If this manual is used in other intensive care nurseries, it must
be revised and adapted to suit the circumstances in those units.
• Guidelines for the initiate management of patients with conditions that require
immediate attention
• Reminders to help them in their daily work
• Detailed instructions for performing procedures
Contributors
Table of Contents
CARDIOVASCULAR
Congenital Heart Disease (T. Bartman, D. Teitel) 95
Patent Ductus Arteriosus (R. Clyman) 99
Shock (V. Londhe) 101
Hypertension (T. Bartman) 103
Cardiac Arrhythmias (J. Kattan, G. Van Hare) 105
HEMATOLOGIC
Anemia (A. Carley, B. Shoemaker) 108
Guidelines for Use of Erythropoietin (R. Phibbs) 111
Polycythemia/Hyperviscosity (C. Retajczyk, C. Miller) 112
Neonatal Coagulation Disorders (A. Carley, B. Shoemaker) 115
Jaundice (C. Botas) 118
Hemolytic Disease of the Newborn (S. Hamrick, R. Phibbs) 121
INFECTIOUS DISEASES
Bacterial Infections (W. Carey) 125
Candidiasis (J. Sherman) 128
Other Congenital & Perinatal Infections 130
(R. Keller, D. Wara, P. Weintrub)
GASTROINTESTINAL
Necrotizing Enterocolitis (K. Ramsdell) 133
Parenteral Nutrition (S. Sniderman, M. Hetherton) 136
NEUROLOGICAL
Neonatal Seizures (S. Miller) 140
Intraventricular Hemorrhage (S. Miller, R. Piecuch) 144
Pain Management and Sedation (J. Partridge) 147
SURGICAL PATIENTS
Neonatal Surgical Conditions (S. Sniderman, H. Lee) 160
Meningomyelocele (N. Gupta) 166
Fetal Therapy (S. Levitt) 168
OTHER
Hydrops Fetalis (R. Phibbs, V. Londhe) 170
Multiple Births (D. Song) 172
Perinatal Substance Abuse (A. Martinez, J. Partridge) 174
Renal Disease (J. Sherman) 178
Neonatal Clinical Physiology Laboratory (J. Kitterman) 181
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Resuscitation of High Risk Infants
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Resuscitation of High Risk Infants
-If naloxone is given, remember that the duration of action is shorter than
narcotics and, therefore, an additional dose of naloxone may be necessary.
•Treat persistent bradycardia in the following sequence
-Ventilate and increase FIO2 if baby does not respond quickly.
-Cardiac compressions using NRP guidelines
-Epinephrine
-NaHCO3 or THAM™ to treat severe metabolic acidosis (pH <7.05 or base
deficit of 15 mEq/L or more) with aims of (1) reversing myocardial failure
and low cardiac output and (2) relieving pulmonary vasoconstriction.
i) Calculation of dose:
Buffer (mEq) = 0.3 x BW (kg) x Base Deficit (mEq/L)
ii) Use NaHCO3 only when infant is receiving adequate assisted
ventilation. With inadequate ventilation, NaHCO3 will worsen
respiratory acidosis (NaHCO3 + H2 → Na+ + H2CO3 → H2O + CO2)
iii) Tham™ is for mixed acidosis as it buffers metabolic acid and lowers
PaCO2. However, it can cause apnea and hypoglycemia.
iv) Infuse buffer at rate of 1 mEq/kg/min.
-Atropine and CaCl2.
-Catheterize an umbilical artery (or vein if unable to get into an artery) for
assessment of pH and PCO2. (See section on Intravascular Catheters, P. 25).
-Drug doses are on placard mounted on wall of Resuscitation Room.
B. Phase II: Evaluation after stabilization: Perform careful physical examination
to detect:
-Major anomalies
-Neural tube defect is easy to miss in a supine infant
-Scaphoid abdomen and respiratory distress should alert one to possibility of
a diaphragmatic hernia
-Dysmorphic features
-Assessment of intrauterine growth
-Signs of infection
•Re-evaluate assisted ventilation
-Be alert for complications of tracheal intubation: dislodgment of tube into
esophagus, inadvertent advancement into right mainstem bronchus.
-Continually assess for changes in pulmonary function
i) Hyperoxia can occur as ventilation and perfusion are better matched.
Manage by decreasing the inspired O2 concentration.
ii) Hypocarbia, due to improved ventilation, can decrease cerebral and
myocardial blood flow. Manage by reducing ventilator rate or PIP.
iii) Hypotension: As lung compliance improves, ventilatory pressures
may become excessive and impede venous return causing hypotension.
Test for this by briefly disconnecting the patient from positive pressure
ventilation; if arterial pressure rises (usually within 5-10 sec), then
reduce airway pressures.
iv) Tension pneumothorax may occur spontaneously or with assisted
ventilation. It leads to hypoxia, hypercarbia, and, if large, it will
obstruct cardiac return and lead to shock.
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Resuscitation of High Risk Infants
C. Phase III: General management. This is the period of time where the mildly
affected infant will begin to improve rapidly and the severely affected infant will
start showing signs of end organ damage.
•Ventilation: Adjust to meet changes in pulmonary function. Give surfactant if
RDS is suspected; RDS, congenital pneumonia and post-asphyxial respiratory
distress may be indistinguishable.
•Use Dopamine to treat post-asphyxial cardiomyopathy. Hypotension may persist
for 1 -2 d and may be distinguished from hypovolemia by ↑ CVP.
•Persistent Pulmonary Hypertension of the Newborn may coexist with asphyxial
cardiomyopathy. Avoid treating PPHN with hypocarbia, as it will ↓ myocardial
(and cerebral) blood flow.
•Coagulopathy is almost always transient. Administer platelets and clotting factors
as needed (see section on Administration of Blood Products, P. 40).
•Hypoglycemia may occur after resuscitation. Treat with continuous glucose
infusion to maintain normal serum glucose; monitor for hypoglycemia and
hyperglycemia
•Fluids and electrolytes
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Resuscitation of High Risk Infants
B. Special situations:
•Twin-to-Twin Transfusion Syndrome (TTTS). Delivery room management will
vary with the clinical picture. Management is based on measurements of their
hemoglobin/hematocrits and arterial and venous pressures.
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Resuscitation of High Risk Infants
-If blood transfusion between twins has been recent, the donor twin will be
anemic and will require volume as with any hypovolemic infant. The
recipient twin will be polycythemic and will require a partial exchange
transfusion to reduce the hematocrit (see section on Polycythemia-
Hyperviscosity, P. 112).
-If blood transfusion between twins has been chronic, the donor twin is
usually SGA and anemic, is likely to need partial exchange transfusion
with PRBCs to treat the anemia because of low tolerance to blood volume
expansion (which occurs with simple transfusion). If the donor twin dies there
is danger of damage to the brain, kidneys and GI tract of the recipient, either
from hypoperfusion or emboli. The recipient twin is usually AGA and may
or may not be polycythemic. Hydrops fetalis may develop in either twin.
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Resuscitation of High Risk Infants
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Intensive Care Nursery House Staff Manual
B. For infants born with any particulate meconium in the amniotic fluid:
1. Assess the infant immediately after birth (in the first 15 sec after birth), before
any drying or stimulation.
2. If the infant is depressed (i.e., absent or depressed respirations, or heart rate
<100/min, or decreased muscle tone in the first 15 sec after birth):
•Immediately perform direct laryngoscopy, before drying or stimulating the infant.
•Suction any meconium that is in the hypopharynx.
•Then intubate the infant’s trachea, apply suction directly to the endotracheal tube
as it is withdrawn from the trachea.
•If meconium is obtained, repeat the intubation and suctioning until little meconium
is recovered or the heart is <60. Do not intubate and suction more than three
times. Then proceed with routine resuscitation.
•With a markedly depressed infant, it may be necessary to give positive pressure
ventilation and proceed with resuscitation despite the presence of some meconium
in the airway. In such an infant, intubate the trachea and suction only one time
before giving positive pressure ventilation.
•Suction catheters inserted through the endotracheal tube may be too small to
accomplish initial removal of particulate meconium; subsequent use of suction
catheters inserted through a tracheal tube may be adequate to continue removal of
meconium.
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Meconium in Amniotic Fluid
3. If the infant is vigorous (i.e., spontaneous respirations, and heart rate >100/min and
good muscle tone in the first 15 sec after birth), use routine resuscitation procedures
as indicated by the infant’s condition. There is no evidence that routine intubation
and tracheal suctioning of vigorous infants is beneficial.
4. Meconium stained infants, who develop apnea or respiratory distress at any time
during resuscitation, should receive tracheal suctioning before positive-pressure
ventilation, even if they had been vigorous initially.
5. There is no evidence that lavage of the trachea is beneficial. Conversely, it may
facilitate movement of the meconium into the distal airways and worsen the infant’s
respiratory status.
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Intensive Care Nursery House Staff Manual
Respiratory Support
ABBREVIATIONS
FIO2 Fractional concentration of O2 in inspired gas
PaO2 Partial pressure of arterial oxygen
PAO2 Partial pressure of alveolar oxygen
PaCO2 Partial pressure of arterial carbon dioxide
PACO2 Partial pressure of alveolar carbon dioxide
tcPCO2 Transcutaneous PCO2
PBAR Barometric pressure
PH2O Partial pressure of water
RQ Respiratory quotient (CO2 production/oxygen consumption)
SaO2 Arterial blood hemoglobin oxygen saturation
SpO2 Arterial oxygen saturation measured by pulse oximetry
PIP Peak inspiratory pressure
PEEP Positive end-expiratory pressure
CPAP Continuous positive airway pressure
PAW Mean airway pressure
FRC Functional residual capacity
Ti Inspiratory time
Te Expiratory time
IMV Intermittent mandatory ventilation
SIMV Synchronized intermittent mandatory ventilation
HFV High frequency ventilation
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Respiratory Support
on FIO2 and flow rate and varies with respiratory rate, tidal volume and the amount of
room air entrained with each breath.
D. FIO2 is measured by portable O2 analyzers, calibrated to display % inspired O2.
ALVEOLAR GAS EQUATION, used to calculate PAO2 (the maximal arterial oxygen
tension that theoretically can be achieved at any given FIO2), is defined as:
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Respiratory Support
4.0
3.5
Et Tube Size
3.0
2.5
10
Lip To Tip Distance (Cm)
1 2 3 4
Figure 1: Top: Solid bars, usual size of tube to be used for infants of the corresponding
birth weight and gestational age; shaded bars, range of bigger and smaller infants in
which that size of tube may be needed on occasion.
Bottom: Distance from the infant's lip to the tip of the tube when the tip is in the
midtrachea. Most endotracheal tubes have numbered centimeter mrks on the sides
indicating the distance to the tip. The appropriate number should be even with the
infant's lip. These are guidelines. There will be some variation among infants.
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Respiratory Support
TRACHEAL INTUBATION: (Many patients die from lack of oxygen, but few die
from lack of an endotracheal tube.)
A. Before attempting tracheal intubation, ventilate the patient’s lungs by mask and and
bag with sufficient O2 to raise PaO2 or SpO2 to normal.
B. With elective or semi-elective intubation and if the baby is hemodynamically stable,
give morphine (0.1 mg/kg) and/or lorazepam (0.05 mg/kg) IV to facilitate intubation,
to prevent increases in intracranial pressure, and decrease stress responses due to
intubation. Monitor blood pressure; even these low drug dosages can produce
hypotension if the infant is hypovolemic.
C. Muscle relaxants are almost never necessary. Do not administer them unless (1)
the infant resists vigorously after sedation has been given and (2) you can effectively
ventilate the patient’s lungs with mask and bag before administering the muscle
relaxant. Succinylcholine may cause cardiac arrest when the serum K+
concentration is elevated or when there is CNS or muscle injury.
D. See Figure 1 for appropriate size of endotracheal (ET) tube. Tube size is
determined by infant’s weight and also by gestational age. Always use an ET tube
with an internal diameter (I.D.) <1/10 of the infant’s gestational age (i.e., if
gestational age is 35 weeks, use a 3.0 I.D. tube, not a 3.5 tube).
E. Use an ET tube that has only an end hole. Do not use tubes with a side hole
(Murphy Eye) close to its tip, as they are prone to occlusion by secretions and
are associated with subglottic stenosis.
F. Laryngoscope: Use Miller #1 blade for term infants and Miller #0 for smaller infants.
G. Empty the stomach to reduce the risk of vomiting and aspiration.
H. Position the patient’s head close to the end of the bed with the head in the “sniffing
position.” Avoid hyperextension or flexion of the neck, since either may occlude the
larynx and make intubation more difficult.
I. During tracheal intubation, place head in neutral position facing straight forward,
insert laryngoscope along right side of the tongue, and move tongue towards the left
side of the mouth. Advance tip of the laryngoscope blade into the vallecula and pull
upward and caudad at a 45° angle. About 90% of the laryngoscope blade should be
within the mouth when the blade is appropriately positioned. This puts the light
where needed and allows for maximal control of the blade. Do not apply pressure on
the gums with the laryngoscope blade, as this may permanently injure the teeth.
J. Hold laryngoscope with thumb and index finger of left hand. Hold chin with ring and
middle fingers, and push on the hyoid bone with small finger of the left hand. This
allows head, hand, and laryngoscope to move as a unit if patient’s head moves, and
prevents pharyngeal injury by the laryngoscope blade. Pushing on the hyoid bone
moves the larynx posteriorly and improves visualization of the larynx.
K. Insert the ET tube under direct vision. While visualizing the larynx, insert the ET
tube through right side of the mouth to the right of the laryngoscope; watch the
ET tube tip pass between the vocal cords. Do not insert ET tube through the
laryngoscope. This blocks your vision and increases the likelihood of esophageal
intubation and injury to the vocal cords. Advance the ET tube until the heavy black
line is at the vocal cords.
L. BE GENTLE! DO NOT USE FORCE!
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Respiratory Support
M. If intubation is difficult and bradycardia +/or cyanosis occur, stop the procedure.
Ventilate the patient’s lungs with mask and bag until color and heart rate return to
normal.
N. Reflex bradycardia is common during tracheal intubation, but heart rate should
return to normal quickly with adequate ventilation of the lungs with oxygen.
O. Figure 2 is a guide for proper positioning of the ET tube. Carefully monitor tube
position until it is securely taped in place. Immediately obtain a chest x-ray to
confirm proper position of ET tube (i.e., tip of ET tube 1 cm above carina).
Important points regarding proper ET tube placement are:
1) Position of infant’s head: Neck flexion advances tube down airway; neck
extension withdraws tube. Try to take x-ray with head in neutral position.
2) Tension on ET tube (With loose tape, tube can move up to 1 cm in or out.
3) Principal focus of x-ray beam.
4) Position and alignment of the clavicles on x-ray.
5) Tip of ET tube should be between T1 and T2 (except with congenital
diaphragmatic hernia, when carina may be more cephalad than normal).
P. The bevel on the ET tube tip must face ventrally, or the tube may obstruct. If the blue
line running lengthwise on the tube is to the left, the bevel will face ventrally.
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Respiratory Support
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Respiratory Support
G. For stridor (usually due to laryngeal edema), give inhalations of the vasoconstrictor,
racemic epinephrine 2.25% (0.2-0.25 mL). For bronchospasm, give inhalations of a
bronchodilator, either metaproterenol (Alupent) 5% (0.1-0.2 mL) or albuterol
(Ventolin) 0.5% (0.1-0.4 mL).
H. When there is progressive or severe respiratory worsening after extubation, reintubate
the trachea early. Do not wait for respiratory failure. After reintubation, it is often
necessary for a time to use higher ventilator pressures (to re-expand areas of
atelectasis) than were needed just prior to extubation. Later, the pressures can be
reduced. It may be useful to obtain a chest x-ray to rule out post-extubation
atelectasis (4-8 h after extubation).
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Respiratory Support
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Respiratory Support
F. High inspiratory flows may injure the lungs. Consequently, it is seldom necessary
to use gas flows >10 L/min. Flows of 6 to 8 L/min are usual; some very small babies
with mild lung disease can be ventilated with 4 L/min. Assess flow rates at start of
PPV, with changes in respirator settings, and if the patient is not improving.
G. Ventilation pressures should be monitored continuously. Guides to adequate
ventilation are normal chest movement and blood gas tensions.
H. Tidal volume is generated by the difference between PIP and PEEP. Changing PIP or
PEEP independently of the other may increase or decrease tidal volume.
I. Oxygenation is primarily a function of FIO2 and PAW , “the area under the curve.”
Figure 3 shows the effects on PAW during a single breath of changing each of the
following: (1) inspiratory flow, (2) PIP, (3) Ti, and (4) PEEP. Note that when
inspiratory flow is increased, PIP is reached earlier and the pressure wave becomes
more “square,” but the durations of Ti and Te do not change. Also, (not shown in
Figure 3) PAW increases when the respiratory rate is increased, because Te decreases.
J. Respiratory rate is altered by adjusting Ti and Te. A very short Te leads to gas
trapping within the lung. The minimal required Te varies with the disease state of
the lungs but usually should not be <0.6 sec with standard mechanical ventilation.
K. Ti is usually set at 0.3 to 0.4 sec (almost always <0.5 sec). Te will then be related to
the respiratory rate. For example, if Ti = 0.4 sec and respiratory rate is 60/min, then
each respiratory cycle lasts 1.0 sec; therefore, Te will be 0.6 sec. If respiratory rate is
30/min with the same Ti (0.3 sec), each respiratory cycle lasts 2 sec and Te will be
1.6 sec. (Note: the “I to E ratio” is calculated from the above. In the first example
I:E = 1:1.5; in the second example I:E = 1:4. Do not manage ventilation by the I:E
ratio. The actual Ti (to facilitate lung inflation), the actual Te (to avoid gas trapping)
and the respiratory rate should guide ventilator management.
L. The time constant (TC) of the lung is a measure of the time necessary for the
alveolar pressure to reach 63% of the change in applied airway pressure. For
example, if Ti = one TC, only 63% of the pressure difference applied by the ventilator
will be equilibrated at the alveolar level, and the delivered tidal volume will be
proportional to the equilibrated pressure. TC is a function of lung compliance and
airways resistance. When lung disease is not uniform throughout the lung, TC will
vary in different parts of the lung. The longer the duration allowed for pressure
equilibration (i.e., during Ti and Te), the greater the equilibration that will occur.
Equilibration of pressures will be 86% complete when Ti (or Te) = 2TC and 95%
complete when Ti (or Te) = 3TC. Further prolongation has little effect. If Ti or Te as
set on the ventilator is not ≥3TC, there may be incomplete delivery of tidal volume
(leading to hypoxemia and/or hypercarbia) or incomplete exhalation (leading to gas
trapping, increased FRC, inadvertent PEEP and hypercarbia). TC varies with the
state of the lung (i.e., type of lung disease). Examples include:
Lung Condition TC (sec)
Normal newborn 0.12
Hyaline membrane disease (HMD) 0.025*
Meconium aspiration syndrome 0.28
*TC increases markedly with recovery from HMD and with surfactant treatment.
Ventilators also have a TC; for most ventilators, complete exhalation requires 0.3 sec.
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Respiratory Support
30
20
15 P AW
PEEP
10
5 Ti Te
0
0 0.5 1.0
Time (Seconds)
Increased PIP
Increased Flow
30 30
Airway Pressure (cm H 2 O)
PIP 25
20 20
15 P AW
15 P AW
PEEP PEEP
10
10
5
5 Ti Te
0
0 0 0.5 1.0
0 0.5 1.0
Time (Seconds)
Time (Seconds)
30
30
PIP
Airway Pressure (cm H 2 O)
25
PIP
Airway Pressure (cm H 2 O)
25
20
P AW 20
15
15
PEEP P AW
10
10
PEEP
5
Ti Te
5
0
0 0.5 1.0 0
0 0.5 1.0
Time (Seconds) Time (Seconds)
Figure 3. Effects of changing different variables on the ventilator. Please see text for
discussion.
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Respiratory Support
FIGURE 4
PIP - PEEP TC
CL
Ti
Flow
Tidal Volume
Te
Ra te
Minute Ve ntilation
PAW
Ox ygenation
Alve olar Ventila tion
PaO 2 a nd Pa CO 2
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Respiratory Support
Raising PEEP to >6-7 cmH2O will rarely increase oxygenation. In fact high
PEEP (and PAW ) may over-distend the lung, leading to increased dead space
(hypercarbia) and right-to-left shunting (hypoxemia) and impair venous return and
cardiac output. In this last case, PaO2 may be normal, but O2 transport and O2
delivery to the tissues will be reduced.
An additional variable is the compression volume of the ventilator circuit, the
amount of gas compressed in the circuit with each breath (i.e., a compression volume
of 5, a common value, means that for every cmH2O pressure generated, 5 mL of gas
will be compressed in the circuit). Compression volume is not important in pressure-
limited ventilation, but is a critical variable for volume ventilation.
N. As lung disease improves (especially after surfactant administration), reduce ventilator
pressures to prevent lung injury. Table 1 gives guidelines to the relationship between
FIO2 and PAW . In general, an infant in lower O2 should be able to tolerate lower
airway pressures.
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Respiratory Support
C. HFV is very effective in removing CO2. Thus, it is easy to hyperventilate the patient.
However, because hypocarbia decreases cerebral blood flow and may cause brain
damage, care must be taken to avoid respiratory alkalosis (i.e., pH>7.5).
D. HFV is less effective at increasing oxygenation. Therefore, compared to
conventional ventilation, a higher PAW is required to achieve equivalent oxygenation.
E. HFV is most commonly used for patients who have air leaks (i.e., pneumothorax or
pulmonary interstitial emphysema), pulmonary hypoplasia or severe pulmonary
hypertension.
F. The oscillator (SensorMedics) pushes gas into the lung during inspiratory phase and
pulls it out during the expiratory phase. This active exhalation may help prevent gas
trapping. This ventilator is effective in large infants. Settings that can be adjusted are:
Rate Usually between 6 and 12 Hz (360 to 720 breaths/min)
Amplitude Similar to tidal volume
PAW Affects oxygenation
Flow Affects inspiratory flow rate
G. The flow interruptor (Infant Star Ventilator) allows a rapid inflow of gas for a brief
period, and then exhalation is passive. HFV can be used with or without a back-up
rate of conventional ventilation. This ventilator is most useful in preterm infants, but
is not usually effective in larger infants.
H. The jet ventilator injects a small volume of gas into the airway through a small tube
connected to the endotracheal tube and entrains gas from the endotracheal tube. Gas
can easily be trapped within the lung. Currently, jet ventilation is not used in the
UCSF ICN.
I. Early studies suggested a higher rate of intracranial hemorrhage in very low birth
weight infants treated with oscillatory ventilation, a finding not seen in later studies.
J. Because of the noise associated with HFV, it can be difficult to detect air leaks or
other lung changes by physical examination and other monitors. Therefore, obtain a
chest radiograph at least daily.
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Respiratory Support
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Respiratory Support
allows it, give 0.1 mg/kg of morphine IV and local anesthesia with 1%
lidocaine (Do not give >5 mg/kg.). Once inserted, connect the chest tube to
suction of 5-10 cmH20 in the suction chamber. In some cases of rapid air
leak, it is necessary to place an anterior chest tube in the midclavicular line.
Avoid inserting this catheter through the breast bud, nipple or areola.
-Suture the chest tube in place and apply a gas-tight dressing.
-When there has been no gas leak for 24 h, turn off the suction on the chest tube.
Do not clamp the tube; leave it to underwater seal. If no gas accumulates
in the pleural space after several hours, rapidly remove the chest tube at end-
inspiration to prevent air from being sucked into the pleural space as the
catheter is removed. Cover the wound immediately with a 2x2 gauze with
bacitracin ointment on it to provide a seal, then close the chest tube hole with
sutures or a clip, and obtain a repeat chest x-ray.
(2) Pneumopericardium
-A pneumopericardium seldom causes problems for an infant.
-Rarely, a tension pneumopericardium causes cardiac tamponade, obstructing
venous return and decreasing cardiac output. When this emergency occurs,
immediately call the Neonatology Fellow or Attending and Cardiology. If
the infant is rapidly worsening, insert a 22 gauge angiocath beneath the
sternum just lateral to the xiphoid aiming towards the left shoulder. Apply
negative pressure as the angiocath is advanced. As soon as air is obtained,
stop advancing the needle or you risk injuring the heart. Withdraw the
needle and leave the angiocath in place connected to a stopcock to allow
evacuation of the gas. If there is time, insert the catheter into the pericardium
under echocardiographic guidance.
-It is rarely necessary to insert a larger tube into the pericardium to remove air.
(3) Pneumomediastinum seldom causes distress or requires treatment in infants. If
it does, have the surgeons insert a mediastinal tube.
(4) Pneumoperitoneum results from perforation of a hollow viscus or from
dissection of air from the mediastinum into the abdomen. Differentiation between
these may be difficult, but can usually be resolved by the following:
-Patients with bowel perforation often appear toxic with discoloration of the
abdomen, leukocytosis and a left shift of the white blood cell count.
-Chest x-ray: A patient with dissection of gas from the mediastinum will
almost always have lung disease with a pneumomediastinum or air seen in
the inferior pleural ligament (“behind the heart”).
-Abdominal paracentesis: With bowel perforation, stool-like material can
usually be aspirated. When pneumoperitoneum results from dissection of
gas from the thorax, only gas can be aspirated.
-Measure PO2 of the aspirated gas. If infant is breathing >30% O2, the PO2
of the gas will usually be >150 mmHg if the gas is from the thorax. With
bowel perforation, PO2 of the aspirated gas is almost always <100 mmHg.
Occasionally, enough gas dissects into the abdomen to restrict ventilation. If this
occurs, insert a catheter or tube into the peritoneal cavity to drain the gas.
D. Chronic Lung Disease most commonly occurs in very preterm infants or after
ventilation with very high pressures and FIO2 (See Chronic Lung Disease, P. 93)
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Intensive Care Nursery House Staff Manual
Intravascular Catheters
ARTERIAL CATHETERS: Most infants admitted to the ICN will need an arterial
catheter for measurement of blood pressure, pH and blood gas tensions. Usually, an
umbilical arterial catheter (UAC) is used. Peripheral arterial catheters are indicated
when:
•Catheterization of umbilical artery was unsuccessful.
•It is desirable to measure pre-ductal PaO2 (i.e., from the right radial a.)
•The UAC has been in place several days or was removed due to thrombus formation.
•The infant is too old to catheterize an umbilical artery.
Figure 1A. Diagram of neonatal arterial system including umbilical artery. (Ao,
aorta; DA, ductus arteriosus; IMA, inferior mesenteric a.; LCCA, left common
carotid a.; LCIA, left common iliac a.; LRA, left renal a.; MPA, main pulmonary a.;
REIA, right external iliac a.; RHA, right hypogastric a.; RUA, right umbilical a.)
Figure 1B. Diagram of the umbilical venous system in a newborn infant. (DV,
ductus venosus; FO, foramen ovale; IVC, inferior vena cava; PS, portal sinus; PV,
portal vein; SVC, superior vena cava; UV, umbilical v.)
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Intravascular Catheters
B. Insertion of UAC:
1. Preparation. Use UAC Insertion tray and appropriate sterile technique including
cap, mask and gloves. Restrain the infant’s limbs. For infants >1500g, use a 5 Fr
catheter; for those ≤1500g, use a 3.5 Fr catheter. Attach a stopcock to the catheter
and fill the system with sterile heparinized flush solution (usually 0.9% NaCl).
Cleanse umbilical cord and adjacent abdomen with iodine solution. Drape the area so
that only the umbilical cord is exposed. Place a cord tie around base of the umbilical
cord and tie loosely. Cut the cord about 0.5 cm above the skin line. If bleeding
occurs, tighten the tie.
2. Insertion of catheter. Hold cord stump gently upright. The umbilical vein is the
single large, thin-walled vessel. The two arteries are smaller, thick-walled, and often
tightly constricted. Gently insert the closed tips of the thin curved forceps into the
lumen of an artery; allow the spring of the forceps to spread the forceps tips apart to
dilate the artery (In extremely LBW infants, it may be necessary to insert only one tip
of the forceps to begin the dilatation). Repeat the process several times until the
lumen is well dilated and the forceps can be inserted into the lumen up to the bend in
the forceps; this is exceedingly important. The most common cause of failure to
catheterize an umbilical artery is inadequate dilatation of the artery.
After the artery is well dilated, insert catheter tip into the lumen and advance the
catheter while directing it towards the pelvis. The catheter may encounter obstruction
at either the level of the abdominal wall or about 5 cm farther, approximately the
level of the bladder. The obstruction can usually be overcome by gentle, steady
pressure for 30-60 sec. Avoid excessive pressure or repeated probing of the artery,
as these may cause arterial perforation. If the obstruction persists, leave the catheter
in place and insert a catheter in the other artery; in most cases, you will be able to
successfully catheterize one of the arteries. When the catheter has passed the point of
obstruction, advance it the appropriate distance for the infant’s size (Table 1). Then,
obtain blood sample for hematocrit, pH and blood gas tensions. Flush the catheter
with heparinized saline and use care to avoid infusing bubbles. Measure arterial
blood pressure. Then secure the catheter.
________________________________________________________________________
Table 1. Guide for distance to insert an umbilical arterial catheter.
Distance to insert umbilical
Birth Weight (g) arterial catheter (cm)*
1,000 7
1,500 8
2,000 9
2,500 10
________________________________________________________________________
*Note that this guide cannot be used for umbilical venous catheters.
3. Securing the catheter: Use a round (non-cutting) needle with 4-0 silk, place one
suture through the wall of the cord and tie a firm knot. Wrap each end of the suture
around the catheter only one time and tie the catheter tightly using a surgeon’s knot; tie
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Intravascular Catheters
firmly but do not occlude the catheter lumen. Be sure catheter cannot slip through the
suture. Apply antibiotic ointment to umbilicus, cover with a dry 2x2 dressing and
secure dressing and catheter with tape.
4. Location of catheter tip. Always verify location of the UAC tip radiographically; it
should be in the abdominal aorta below the 3rd lumbar vertebra (L-3) and above
the aortic bifurcation (usually, bottom of L-4). This will ensure that the tip is below the
origin of the inferior mesenteric and renal arteries but still in an area of relatively high
blood flow. If the UAC is advanced farther into the aorta and into the thorax, the tip
will almost always pass through the ductus arteriosus into the pulmonary artery. This
will lead to errors in treatment, because PaO2 and blood pressure in the pulmonary
artery are almost always lower than in the aorta. After completing the catheterization,
examine the infant’s legs for evidence of decreased femoral arterial blood flow
(blanching, mottling, decreased or absent femoral pulse). If femoral flow is
decreased, remove the catheter.
5. Catheter maintenance. Maintain a constant infusion of heparinized fluid through the
UAC. Heparin concentration should be 1 unit/mL. Examine the legs daily and remove
UAC if there is evidence of decreased femoral blood flow. Also, remove the UAC if
there is damping of the arterial wave form (See below under Blood Pressure).
C. Complications of umbilical arterial catheters are listed below and can cause
serious, and sometimes fatal, consequences:
-Ischemia due to obstruction of blood flow to the legs (see above). When UAC tip is
above L-3, there may be occlusion of the inferior mesenteric a. leading to bowel
ischemia resulting in necrotizing enterocolitis (NEC). Do not feed an infant with a
UAC. If abdominal distension or other signs of NEC occur, remove the UAC.
-Thrombosis, the most common complication of a UAC, may cause damping of the
arterial tracing, NEC, renal insufficiency, hypertension (renovascular or secondary to
aortic obstruction) or decreased blood flow to the legs. If there are signs of
thrombosis, remove the UAC.
-Emboli occur from small bubbles inadvertently infused into the UAC (e.g., with
flushing) or particulate matter from a thrombus on the UAC. If signs of emboli
occur, remove UAC. Because packed RBCs infused into a UAC have caused
infarcts of the spinal cord with resultant paralysis, do not give packed RBCs
through a UAC.
-Vasospasm: If the leg blanches, warm the other leg to induce reflex vasodilatation. If
improvement does not occur, remove the UAC.
-Hemorrhage will occur if UAC is accidentally disconnected from stopcock or tubing.
-Vascular perforation may occur if excessive pressure is used to insert the UAC.
Massive intra-abdominal hemorrhage may result.
-Hypoglycemia can occur if the UAC tip is above the recommended site. The infusion
of glucose may stream into the pancreatic a. via the celiac axis, causing
hyperinsulinemia and resultant hypoglycemia.
-Infection is rare with arterial catheters and can usually be prevented by keeping the
cord covered with antibiotic ointment and a dry dressing.
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Intravascular Catheters
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Intravascular Catheters
VENOUS CATHETERS
UMBILICAL VENOUS CATHETERS: In most circumstances, arterial catheters are
safer and more useful than venous catheters. However, in some cases, an umbilical
venous catheter (UVC) is desirable (e.g., for exchange transfusion, measurement of
central venous pressure).
A. Insertion of UVC: Preparations are similar to those for UAC. The vein is the large,
thin-walled vessel in the cord.
•Remove any visible clots in vein with forceps.
•Connect catheter to pressure transducer, before inserting it into the vein.
•Insert catheter using only gentle pressure.
•Never open UVC to atmospheric pressure as this may result in air embolism.
•While continuously measuring pressure, insert catheter into umbilical vein.
B. Location of UVC Tip. Figure 1B shows the relevant anatomy. Note the several
possible locations for the UVC tip, including umbilical vein, portal vein, portal sinus,
right atrium, left atrium, left ventricle, pulmonary vein and SVC (rare).
1. The preferred location is beyond the ductus venosus in IVC or low right atrium.
2. Placement of UVC tip in the portal system is undesirable for the following reasons:
•Portal venous pressure is higher than central venous pressure, but by a variable
amount and thus gives no useful information about the cardiovascular system.
•Infusion of hypertonic solutions (e.g., 10% glucose, NaHCO3) into the portal
system may thrombose the portal vein.
•Exchange transfusion with the UVC tip in the portal system may cause necrotizing
enterocolitis.
3. If the UVC tip is advanced farther into the right atrium, it almost always passes
through the foramen ovale into left atrium. If advanced farther, the UVC tip will enter
either the left ventricle or a pulmonary vein. Because of the risk of systemic emboli,
do not allow the UVC tip to remain in the left side of the heart.
4. It is very unusual for a UVC to advance up into the SVC and into a jugular vein.
C. Placement of UVC. The location of the UVC tip cannot be determined by the
distance the catheter has been inserted. It must be localized using pressure
measurements, measurements of PO2, and by radiograph:
1. Advance UVC while continuously measuring pressure. As the tip passes through the
ductus venosus, the pressure will decrease and the wave form will resemble an atrial
pressure tracing (See Figure 2). If you are unsure if the tip is in the thorax stimulate the
infant to take a deep inspiration or cry. If the UVC tip is in the thorax, there will be a
negative pressure during spontaneous inspiration (Figure 2A). Note that the pressure
never goes below atmospheric when the UVC tip is in the portal system (Figure 2B).
2. When the UVC tip has entered the thorax, take a blood sample to measure PO2; If PO2
is >50 mmHG, it is likely that the UVC tip is in left atrium, pulmonary vein or left
ventricle. If it has been advanced into LV, a ventricular pressure wave will be seen.
Withdraw UVC until tip is in right atrium (Blood will appear less pink and pressure
tracing will have a dominant “a” wave; see below).
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Intravascular Catheters
Figure 2. Venous pressures measured through an umbilical venous catheter with tip
in the right atrium (A) and in the portal sinus (B). A. With the catheter tip in the
right atrium, there are negative pressure deflections (I) during spontaneous inspiration.
When the infant takes a deep inspiration or sigh (S), the pressure goes well below
atmospheric pressure to about 10 mmHg. B. With the catheter tip below the diaphragm
in the portal venous system, the mean pressure is higher than central venous pressure,
the pressure goes slightly positive during inspiration (I) and never goes below zero
(atmospheric pressure).
3. When catheter has been localized to right atrium by measurements of pressure and
PO2, secure catheter and obtain a chest radiograph to confirm the position.
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Intravascular Catheters
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Intravascular Catheters
avoid displacement. Apply digital pressure on the vessel above the insertion
site to minimize bleeding.
-Breakaway Technique: Ensure stability of introducer needle while advancing
catheter into vein.
•Insert catheter into sheath/introducer needle and advance slowly to desired depth
using forceps. Loosen tourniquet after catheter is advanced beyond tip of
introducer. Flush intermittently to facilitate catheter advancement. Do not
withdraw catheter while needle is in the vein, as this may shear off catheter.
During insertion, observe ECG for dysrhythmia or bradycardia.
•Once catheter has been advanced to proper position, withdraw sheath/introducer
needle while maintaining pressure on vein just beyond the needle tip to prevent
inadvertent withdrawal of the catheter. Continue to apply pressure over the
needle insertion site after the needle/sheath has been removed, as bleeding is
common and often lasts for a few minutes.
•After with drawing needle or sheath:
-Peel-Away technique: Split the introducer sheath and peel it away from the
catheter, using care to maintain catheter position.
-Breakaway technique: With the wings of needle facing downward, grasp each
wing between thumb and index finger. Snap the wings upward until the plastic
portion is completely separated. Peel away from the catheter allowing the
catheter to fall down. The needle is not designed to break completely.
•Measure length of the catheter remaining outside of the skin. Subtract this
measurement from total length of catheter. The remaining is the length of
catheter in the patient and should approximate the previously calculated desired
catheter insertion length. Make adjustments in catheter position as necessary.
•To ensure catheter patency, aspirate the catheter with a heparinized saline filled
syringe to visualize blood return and then gently flush.
•Apply steri-strips over puncture site to secure catheter. Refer to nursing procedure
for dressing guidelines.
(d) Documentation: Write a procedure note describing site, catheter type and length,
tip location, patient response, and complications (if any). This information should be
recorded on the PICC documentation form.
(e) Radiographic confirmation of catheter tip location: Use radio-contrast material
(e.g., Omnipaque 180™) to visualize the catheter. Use 0.1 mL of contrast for
Vygon™ 27 gauge, and NeoPICC™ 1.9 and 2.8 Fr catheters . Immediately before the
x-ray cassette is exposed, instill the contrast at the hub of the catheter, injecting
slowly with a 3cc syringe. Prior to exposing the x-ray, place the involved upper
extremity in a position of maximal abduction, or the involved lower extremity in a
fully flexed position. After radiograph has been taken, withdraw the contrast and
gently flush the catheter with saline (0.9%) before reconnecting IV tubing. If the tip
of the catheter is still difficult to visualize, repeat the film with a slightly oblique view
with the side of catheter placement elevated. Monitor catheter tip placement with
weekly (q Monday) radiographs.
(f) Proper location of catheter tip: For catheters inserted through arm or scalp vein,
tip should be in SVC just above right atrium. For catheters inserted through a leg
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Intravascular Catheters
vein, catheter tip should be in IVC, just below right atrium. The tip of a PICC
should never be in the heart, because of the risk of perforation and cardiac
tamponade.
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Intravascular Catheters
0.9% NaCl + + +
0.45% NaCl + + +
5% Glucose + + +
6 to 12.5% Glucose + + 0
Ringer's Lactate + + 0
Calcium + +4 0
Antibiotics and + + 0
Other Medications
________________________________________________________________________
(+ = acceptable; 0 = unacceptable)
1 Do not give intravenous alimentation with lipids and/or amino acids via UVC or UAC.
2 This guide assumes that it is known that the tip of the UVC is in IVC or right atrium
and not in the portal system or the left side of the heart.
3
This assumes that it is known that the tip of the UAC is in proper position.
4
Give calcium into UAC as a push only in emergency situations.
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Intensive Care Nursery House Staff Manual
Blood Pressures
Measure intravascular pressure continuously in all infants who have:
•Arterial catheters (umbilical or peripheral)
•Umbilical venous catheters
•Central venous catheters (Unless the infant's cardiorespiratory status is stable and
catheter was inserted only for intravenous alimentation.)
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Blood Pressures
•Excessive ventilatory pressures that impede venous return to the heart. To test for this,
briefly disconnect the ventilator from the endotracheal tube. If ventilatory pressures are
excessive, arterial pressure will rise in <10 sec.
•Marked alkalosis and hypocarbia
•Myocardial failure due to:
-Asphyxia -Cardiomyopathy
-Hypocalcemia -Cardiac arrhythmias
-Congenital heart disease (left sided obstructive lesions)
•Drugs (e.g., PGE1, nitroprusside, isoproterenol, vancomycin [if infused
rapidly])
(b) Hypertension may be caused by:
•Hypercarbia, moderate asphyxia
•Polycythemia
•Renovascular disease
•Drugs (e.g., dopamine, epinephrine, phenylephrine)
•Hypervolemia usually does not cause systemic hypertension, but may cause
pulmonary hypertension.
•Pain
2. Abnormal arterial pulse pressures
(a) Narrow pulse pressure may be caused by:
•Damping of pulse wave (See below)
•Tension pneumothorax or other severe air leak
•Improper catheter position
•Congenital heart disease (e.g., coarctation of aorta, aortic stenosis)
•Myocardial failure
•Shock
(b) Wide pulse pressure may be caused by:
•Patent ductus arteriosus
•Arterio-venous malformation
•Truncus arteriosus
•Vasodilator drugs
B. Damping of the blood pressure tracing is due to decreased frequency response of the
system. It abnormally narrows the pulse pressure and should be suspected when the dicrotic
notch is not visible on the blood pressure tracing. An example of damping of an arterial
pressure tracing is shown in Figure 3. Damping can be caused by:
•Air bubbles in the catheter system or transducer
•Blood in the catheter system
•Clot in the catheter
•Thrombosis of the artery
•Kinking of the catheter (with peripheral arterial catheters)
•Abnormal position of catheter tip (e.g., UAC pointed down a femoral a.)
•Damping of venous blood pressure also can occur (e.g., when UVC tip is against the
atrial wall or wedged in the liver or a pulmonary vein)
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Blood Pressures
80 Damped Normal
40
0
Time (seconds)
Figure 3. Damped blood pressure tracing measured through an umbilical
arterial catheter in a newborn infant. The damping was caused by an air bubble in
the catheter system. When the bubble was removed, the fidelity of the tracing
improved and both the anacrotic and dicrotic notches could be detected.
Damping will affect systolic, diastolic and pulse pressures. However, in most cases, the
mean pressure will be accurate.
1. If there is damping of an umbilical arterial or venous pressure wave form and the
damping cannot be corrected by removing blood and/or bubbles from the system,
remove the catheter. Do not flush a damped catheter. This may cause embolism
with disastrous consequences.
2. Damping of the pressure wave from a peripheral arterial catheter is less serious and
can be tolerated if the catheter samples well. Do not vigorously flush a damped
peripheral arterial catheter because of the risk of retrograde emboli.
CENTRAL VENOUS PRESSURE (CVP): In most cases, the trend in CVP (over
several minutes to hours) is more helpful than the absolute value of the CVP. CVP may
be difficult to interpret because it is affected by several factors.
A. Increased CVP may be caused by:
•Hypervolemia •Myocardial failure (from any cause)
•Excessive ventilatory pressures •Grunting respirations
•Tension pneumothorax •Pleural effusion
•UVC tip in portal system (i.e., not central venous)
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Blood Pressures
C. Location of UVC Tip. It may be difficult to know if the UVC tip is in right or left
atrium even with radiographs and measurements of PO2. Comparison of the CVP
tracing with the ECG tracing can be helpful. In right atrium, the “a” wave is
dominant; in left atrium and pulmonary veins, the “v” wave is dominant (Figure 4).
______________________________________________________________________
ECG
a v a v
Pv
Figure 4. Relationships of right and left atrial pressures to ECG in newborn infants.
a, atrial “a” wave; v, atrial “v” wave; ECG, electrocardiogram; Pv, venous pressure
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Blood Pressures
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Intensive Care Nursery House staff Manual
IRRADIATION of all blood products is routine for ICN patients. This is to prevent
graft versus host disease in immuno-compromised patients. Because some patients
require transfusion before their immune status is known, all blood products are irradiated.
CMV NEGATIVE whole blood, PRBCs, platelet and white blood cells should be given
to immuno-compromised patients to prevent tranfusion acquired CMV infection.
However, because of the limited availability of CMV blood products, CMV negative
products are given only to infants with birth weight <1.5 kg (and <4 months of age) and
infants at high risk of being immune-deficient (e.g., congenital heart disease).
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Blood Products
•Mild (e.g., nasal CPAP, head hood O2): transfuse for Hct <30%
•Apnea of Prematurity: transfuse for Hct <30% and worsening apnea (see section
on Apnea, P. 91).
(2) Stable, growing preterm infant: Consider transfusion
•If Hct <20% and reticulocyte count <100 x 109/L.
•If no weight gain for several days with adequate caloric intake, Hct <28% and
other signs consistent with anemia (e.g., tachycardia, tachypnea)
(3) Infant with sepsis if Hct <30%
(4) Infants with symptomatic heart disease: transfuse to keep Hct 40-45%. With large
left-to-right shunt, maintain Hct in 50-55% range. Except in emergency, discuss
with Cardiology before transfusing any infant with heart disease.
Transfusion volume: PRBCs must be infused within 4h of their release from Blood
bank. Therefore,
A. Infuse 15 mL/kg over 1-3h or
B. Infuse 10mL/kg over 1-3h, check Hct 1h later and, if needed, give another 10 mL/kg
from same quadpack (same donor).
C. Special Considerations
•For an infant unlikely to tolerate volume overload (e.g., symptomatic PDA,
Chronic Lung Disease), use B (above) and give furosemide (1mg/kg IV) after 1st
transfusion.
•For all transfusions, check Hct 1-4 h after completion of the transfusion.
•If PRBCs are being given for volume resuscitation, give transfusion more rapidly,
over 15-30 min.
2. Platelet transfusion: See section on Bleeding Disorders (P. 115) for causes of
thrombocytopenia
•¼ unit of platelets/kg will raise the platelet count more that 50,000
•give dried platelets for infants with concern for volume overload
Indications for platelet transfusion:
•Stable infant with platelets <20 x 109/L
•Active bleeding with platelets <50 x 109/L
•Platelets <50 x 109/L in a “sick” infant (e.g., preterm infant on mechanical ventilation)
Transfusion volume varies with condition. Usual starting volume is 10 mL/kg of
platelets. For a term infant with birth weight >2.5 kg, transfuse 1 unit of platelets.
3. Plasma components are usually given for specific clotting deficiencies. See section
on Bleeding Disorders (P. 115) for indications. Note: Fresh frozen plasma is to be used
only for hemostasis. Treatment of hypovolemia is discussed in the section on Neonatal
Shock (P. 101).
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Exchange Transfusion
2. Technique: With the push-pull method, use increments of 5 mL/kg. Small increments
are safer and just as efficient as larger ones, provided that you clear the donor blood from
the dead-space of the catheter. Do this at the end of each infusion increment by
withdrawing 2 mL of blood from the catheter into the syringe and then reinfusing it.
During the procedure, the operator(s) must call out the volume in and out with each
infusion and withdrawal (e.g., “ten in - ten out”). A 3rd person must keep a written timed,
running record of each infusion and withdrawal and of cumulative volumes to be sure that
the volumes infused and withdrawn are equal.
Take 45-60 min to perform a 2 volume ExTx in a vigorous baby and longer in a sick one.
If the infant is receiving O2 or assisted ventilation, measure pH, PaCO2 and PaO2
frequently (e.g., q 100 mL). You often will need to increase FIO2 during the ExTx.
3. Important reminders:
•Monitor ECG, blood pressure, O2 saturation, transcutaneous CO2 and temperature
during ExTx.
•Measure pH at mid-point and at end of ExTx (more frequently in a “sick” baby.
•Measure glucose and electrolytes at end of ExTx, and glucose at 10, 30, 60 min later.
•Warm blood to 34-35º C. Warming blood to >37º C causes hemolysis.
•Agitate the unit of donor blood q 10-15 min so that cells do not settle.
•ExTx does not significantly ↓ plasma gentamicin level; do not give an extra dose.
4. Complications of ExTx:
Problem with Effect on
Donor Blood Infant Prevention or Treatment
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PHYSIOLOGY: Blood is drained from the patient to an external pump which pushes the
blood through a membrane gas exchanger (for oxygenation and CO2 removal) and
warmer and returns the blood to the patient’s circulation. The method requires heparin
anticoagulation of the patient, that is managed by frequent measurements of activated
clotting time (ACT). Various devices monitor pressures, flow, and temperature of the
ECMO blood and gas circuits, as well as physiological variables in the patient. ECMO
can be either:
(a) Veno-arterial (VA), in which blood is drained from right atrium (via a right
internal jugular venous catheter) and is returned to the thoracic aorta (via a right
carotid arterial catheter). VA-ECMO provides cardiac as well as pulmonary
support.
(b) Veno-venous (VV), in which blood is drained from right atrium (via side holes of a
double lumen catheter) and returned to the right atrium through the end hole of the
catheter which is directed towards the tricuspid valve. VV-ECMO requires good
cardiac function and avoids cannulation of the carotid artery.
Cannulation for ECMO is done by a Pediatric Surgeon and patient management is by
Neonatology. With both forms of ECMO, the ventilator settings are decreased to allow
recovery of lungs, but generally PEEP is maintained at higher pressure (e.g., 8 cm H2O)
to prevent atelectasis.
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ECMO
MANAGEMENT: Initial settings are aimed at bypass of ≥50% of cardiac output and are
adjusted to maintain adequate oxygenation, blood pressure and acid-base status. With
cardiac failure, VA ECMO is the preferred method. Because of recirculation, VV ECMO
cannot usually support >50% of cardiac output, which may limit the ability to adequately
oxygenate the patient. Patients on ECMO require frequent measurements of pH and
blood gas tensions and various laboratory tests, as well as frequent transfusions with
packed RBCs and platelets. Meticulous attention to all aspects of a patient’s condition is
essential. The infants are sedated, but usually do not require paralysis while on ECMO.*
Allowing the patient to move facilitates neurological assessment. As patient improves,
ECMO support is gradually reduced. Patient is decannulated when able to tolerate
minimal ECMO support on low to moderate ventilator settings. The duration of ECMO
treatment is usually limited to 7-10 d for neonatal respiratory diseases, but longer
treatment may be needed for newborns with diaphragmatic hernia and cardiac disease and
in older children.
*One exception includes patients with congenital diaphragmatic hernia prior to
operative repair. They are usually kept paralyzed to prevent air swallowing which dilates
the bowel, complicates the operation and interferes with pulmonary function.
COMPLICATIONS:
-Hemorrhage (pulmonary, GI, surgical site) -Cardiac dysrhythmia
-CNS damage (bleeding or infarction) -Renal failure
-Seizures (metabolic or CNS causes) -Hyperbilirubinemia
-Fluid retention and severe edema -Sepsis
Note: The above comments apply primarily to neonatal patients. Occasionally, older
infants and children, and even some adult patients, benefit from ECMO treatment.
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Intensive Care Nursery House Staff Manual
I. ADMISSION PROCEDURES:
A. Outborn infant transported from another facility:
1. Arterial pH and blood gas tensions while infant is in transport incubator
2. Weigh infant and transfer to ICN radiant warmer bed.
3. Apply monitors:
-Leads for cardio-respiratory and temperature monitors
-Probe for Pulse Oximeter (oxygen saturation)
-Skin electrode for continuous measurement of transcutaneous CO2
4. Measure and record vital signs and do physical assessment.
5. Connect IV fluids.
6. Obtain blood for the following:
-Hematocrit, CBC with differential, platelet count, and type, Rh & antibody
screen
-If infant’s age >12h, blood is also sent for electrolytes, calcium, BUN, creatinine,
and bilirubin (total and direct)
B. Inborn infant admitted from Resuscitation Room (Set-up Room)
1. Plug warmer bed into electrical outlet
2.Apply monitors:
-Leads for cardio-respiratory and temperature monitors
-Probe for Pulse Oximeter (oxygen saturation)
-Skin electrode for continuous measurement of transcutaneous CO2
3. Measure and record vital signs
4. Connect IV fluids
5. Unless obtained during resuscitation, send blood for Hematocrit, CBC with
differential, platelet count, and type, Rh & antibody screen.
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Routine Nursing Procedures
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Intensive Care Nursery House Staff Manual
An isolation incubator provides warm ambient air at about 50% humidity, thereby
reducing convective, and to a lesser extent, evaporative and conductive losses. Its
relatively warm double-walls reduce radiant losses. A clear plastic wrap over the whole
body reduces convective and evaporative losses but could obstruct the nose and mouth.
Use this only in intubated infants. Clothing, particularly a hat, further reduces heat
loss. Radiant heaters provide effective heating but create high evaporative water losses.
Infants <36 weeks gestation should be placed in an incubator or under a radiant warmer
with temperature probe on the skin. Set the incubator temperature at neutral thermal
environment for infant's weight and gestational age (i.e., that environmental temperature
at which oxygen consumption and caloric utilization are lowest). If an infant <1,500 g
must be under a radiant warmer to allow easier access, cover the infant with a plastic
wrap (if intubated) and cover the head with a hat.
Temperature control for newborn infants is important to avoid the excess stress which
hypo- or hyperthermia imposes on a newborn. To provide a neutral thermal environment,
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Health Care Maintenance
the incubator temperature should be kept at the temperatures shown in the following
tables.
When the incubator is opened for procedures, use a portable overhead warmer with
temperature probe and/or a Portawarmer™ to prevent hypothermia.
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0 5 10 15 20 25 30 35 40
Gestation (weeks)
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Feeding of Preterm Infants
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Feeding of Preterm Infants
Volume of
Gestational first feed Rate of feeding
Age (weeks) (cc/kg) Frequency advance
FORTIFYING FEEDINGS not only provides mores calories but also improved intake
of calcium, phosphorus and protein. Fortify feedings (breast milk and formula) as
follows:
-When infant is tolerating ≥100 cc/kg/d, feedings may be fortified to 22 cal/oz.
-When infant has been tolerating ≥150 cc/kg/d for at least 2d, feedings may be
fortified to 24 cal/oz.
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Feeding of Preterm Infants
If feedings have to be stopped for any of these reasons, notify the Neonatology Fellow
and/or the Attending Physician, so that they can follow the infant’s condition with you.
If there is any doubt about how well an infant is tolerating feedings, it is best to hold
feedings, evaluate the infant and discuss the case with the other members of the
team.
Experienced ICN Nurses are experts at feeding small preterm infants and are
valuable resources for advice on feeding problems.
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Term infants
Feeding Vitamin Supplement q d Iron Supplement q d
Maternal breastmilk Consider Vitamin D 200 IU by 4-6 months
Term Formula with Iron none none
All infants
A fluoride source is needed by 6 months of age (corrected GA for preterm infants).
*Preterm infants on full feeds should be fed fortified breastmilk or preterm formula, unless there are clinical reasons to
use another formula or not to fortify breast milk.
ψEnfamil Human Milk Fortifier (EHMF) contains iron
‡Assumes infant will receive iron 2 mg/kg/d in formula at volume to meet 120 kcal/kg/d.
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BODY COMPOSITION at birth varies with gestational age and body weight.
Examples are shown in the table below. (BW, body weight)
Gestation Body Total Body ECW Body
(weeks) Weight (g) Water (%BW) (%BW) Fat (%BW)
24-27 <1,000 85-90 60-70 0.1-2.5
28-32 1,500 82-85 50-60 3.3-5.5
36-40 >2,500 71-76 ~40 9-16
After birth, infants lose weight due to loss of ECW, and this is proportionately greater in
smaller, less mature infants. Weight loss after birth reflects this difference in ECW; term
infants normally lose up to 5% of their body weight, whereas very immature infants may
lose up to 10-15%. Thus, fluid replacement must be adjusted accordingly.
INSENSIBLE WATER LOSS (IWL) is greatest in the smallest and least mature infants
due to high surface area to body mass ratio and to immature, water-permeable skin.
Estimated IWL in the first few days of life are:
Body Insensible water loss (mL/kg/d)
Weight (g) In Radiant Warmer In Incubator
<1,000 100-150 75-100
1,000-1,500 75-100 50
1,500-2,000 50 25-50
>2,000 50 25-50
Phototherapy can increase IWL by 25-50%. IWL may exceed urinary output in smaller
infants but, unlike urine output, IWL cannot be measured directly. However, IWL must
be estimated in order to plan appropriate fluid management. IWL can be estimated by:
IWL = Fluid intake - Urine output + weight loss (or – weight gain)
(e.g., 24-hour totals = intake 90 mL, urine output 60 mL, and weight loss 55 g.
Therefore, IWL = 90 - 60 + 55 = 85 mL)
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Fluids and Electrolytes
Some SGA infants have increased IWL and, therefore, need increased fluid intake to
compensate for this (see section on Intrauterine Growth Retardation, P. 69).
INITIAL FLUID ADMINISTRATION: Guidelines for the first few days of life are:
•For larger infants (i.e., >1,250 g) start at 60 mL/kg/d of D10W. If estimated ILW is
high (e.g., extreme prematurity, abdominal wall defect), start fluids at higher rate, 80-
100 mL/kg/d.
•Normal glucose utilization rate in a newborn is 4-8 mg/kg/min. Initial glucose
administration rate should be in that range.
•Extremely premature infants (23-26 weeks gestation) under radiant warmers may
occasionally require more than 200 mL/kg/d during the first 2 to 3 days of life.
•Increase IV fluid rate if weight loss is >expected, urine output is low, urine specific
gravity is rising, and/or serum sodium concentration [Na+] is rising.
•Conversely, decrease IV fluid rate if serum [Na+] is falling, weight did not decrease
appropriately or actually increased.
•Proper fluid management requires accurate determination of urinary output in mL/kg/h.
In the first 24h after birth, urine output may be very low (or even absent) in normal
newborns. After the first day, urine output should be >1 mL/kg/h.
•If you make a major change in the rate of fluid infusion, you must also change the
glucose concentration proportionately to maintain a constant rate of glucose delivery
and prevent hyperglycemia (and osmotic diuresis) or hypoglycemia.
•Fluid requirements gradually decrease by day 5-6 as skin permeability decreases.
1. General guidelines:
•All infants receiving only IV fluids should have daily measurements of electrolytes for
the first few days of life. The frequency can be reduced as condition stabilizes.
•For infants <750 g, measure electrolytes within 12h of birth to have a baseline, so that
adjustments in fluid intake can be made as serum sodium changes. In these extremely
preterm infants, significant hyperkalemia may develop in the first 48-72h.
•Measure BUN and creatinine initially and at least every other day until stable, then
weekly until feedings are well established.
•Measure magnesium in first few hours after birth if mother had received magnesium.
Suggested frequency of measurements of electrolytes, including calcium for infants
receiving only IV fluids:
<750 g q8-12 h x 3-4d, then daily
750-1,500 g q12 h x 3-4 days, then daily
>1,500 g daily
2. Sodium:
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•Do not add Na+ to IV fluids on the first day; wait until day 3-4 when [Na+] begins to fall.
Na+ is usually given as NaCl, but Na-acetate may be used to decrease metabolic
acidosis from renal bicarbonate wasting in ELBW infants.
•Usual maintenance for Na+ is 2-4 mEq/kg/d.
3. Potassium (K+):
•Do not add K+ to IV fluids for the first few days after birth, until urine output is well
established and serum K+ level starts to decline. K+ may be given as KCl or K-acetate.
•Usual maintenance for K+ is 1-3 mEq/kg/d.
4. Calcium (Ca++):
•Ca++ should be started on the first day after birth especially in infants who are preterm,
SGA, asphyxiated, septic, and post operative, and infants of a diabetic mother.
•Ca++ may be added to the IV solution infusing through central catheters after the
location of the catheter tip has been verified radiographically to be in proper
position. This includes umbilical arterial and venous catheters and central venous
catheters (see section on Intravascular Catheters, P. 25).
•Ca++ should not be added to IV solutions infusing in peripheral veins because
extravasation of Ca++ containing solutions may cause severe sloughing of skin. If
peripheral IV access is being used, Ca++ should be given as an intermittent bolus over 5
to 15 minutes while watching the IV insertion site to ensure that fluid is not infiltrating
into the tissues.
•Usual maintenance for Ca++ is calcium gluconate 200-400 mg/kg/d.
•Usual intermittent dose is calcium gluconate 50-100 mg/kg IV q6h.
ELECTROLYTE ABNORMALITIES:
1. Hyponatremia is defined as serum [Na+] <130 mEq/L. Hyponatremia may cause
hypotonia, apnea, and, if acute and severe, seizures. In the first few days after birth,
hyponatremia usually indicates fluid overload (i.e., dilutional hyponatremia). After
the first week, it may be either dilutional or indicate a true deficit of total body Na+.
A. Dilutional hyponatremia is usually accompanied by weight gain or absence of
expected weight loss and may be secondary to:
•Renal dysfunction with ↓ urine output and usually a low urine specific gravity.
•Excessive water intake, often with high urine output and low specific gravity.
•Congestive heart failure with ↓ urine output and ↑ specific gravity.
• ↑ extracellular fluid volume (e.g., water retention due to sepsis, prolonged use of
muscle relaxants).
•Syndrome of inappropriate secretion of anti-diuretic hormone (SIADH) with low
urine output and high urine specific gravity. This condition is rare.
Dilutional hyponatremia should be treated primarily by fluid restriction. However,
if serum [Na+] is <120 mEq/L, the baby may require additional Na+ as well.
B. Sodium deficiency may be accompanied by weight loss and may be caused by:
•Diuretic administration •Renal Na+ losses
+
•Low Na intake •Osmotic diuresis from hyperglycemia
•Gastrointestinal Na+ losses
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Treat Na+ deficiency by treating the underlying condition and increasing Na+
intake. If hyponatremia is caused by diuretic treatment for chronic lung disease,
consider decreasing the diuretic dose or giving the diuretic every other day instead
of daily, and consider tolerating lower serum [Na+] (e.g., 125-130 mEq/L). Total
body deficit of Na+ can be calculated by:
Na+ deficit (mEq) = (desired [Na+] - current [Na+]) x 0.8 x body weight (kg)
(0.8 x body weight is the volume of distribution for Na+)
C. Symptomatic hyponatremia: (e.g., seizures or [Na+] <120 mEq/L). Calculate
Na+ deficit to raise [Na+] to 125 mEq/L and give as 3% NaCl (0.5 mEq/mL) over
3-6h. Correct remaining deficit over next 24h.
D. Asymptomatic hyponatremia: Calculate total deficit of Na+ and give ½ over 6-
8h and the rest over the next 24h, as Na+ added to IV fluids.
2. Hypernatremia (serum [Na+] >150 mEq/L) may cause hyperexcitability and
hyperreflexia. Severe hypernatremia (serum [Na+] >160 mEq/L) may cause permanent
CNS damage. Hypernatremia is usually secondary to excess Na+ intake or negative water
balance. Usually in a newborn, excessive Na+ intake leads to excess total body water,
and, thus, serum [Na+] is normal. When hypernatremia is due to excessive Na+ intake,
restrict Na+ intake and consider administering diuretics. When due to water deficit, it is
associated with weight loss and should be treated by increasing free water to correct
negative water balance slowly. In babies with meningitis, hypoxic ischemic
encephalopathy or severe intracranial hemorrhage, consider diabetes insipidus, which
should be treated initially by increasing free water administration. Rapid correction of
hypernatremia may cause seizures and permanent neurodevelopmental sequelae.
Therefore, do not lower serum [Na+] more rapidly than 10 mEq/L q12h.
3. Hypokalemia (serum [K+] <3 mEq/L) may cause ileus, arrhythmia (unusual unless
[K+] is <2.5 mEq/L), and altered renal function. Hypokalemia may be caused by:
•↑ K+ loss from diuretics, diarrhea, renal defect
•Inadequate K+ intake
•↓ extracellular K+ secondary to metabolic alkalosis.
If the hypokalemia is secondary to metabolic alkalosis, correct alkalosis before
considering increasing K+ intake. For other causes of hypokalemia, increase K+ in daily
maintenance fluids. K+ must never be given as a push or bolus infusion because of
the risk of serious cardiac arrhythmias. In extreme emergencies, K+ can be given as a
rapid infusion, but give no more than 0.3 mEq/kg over 20 min.
4. Hyperkalemia (serum [K+] >6 mEq/L) may cause lethal arrhythmias, especially
ventricular fibrillation. Early EKG changes of hyperkalemia include peaked T waves and
widening of the QRS complex. The most common cause of high serum [K+] is hemolysis
of the specimen. When an abnormally elevated [K+] is reported by the Laboratory, send
a repeat sample for stat [K+] measurement before starting treatment unless the EKG
indicates hyperkalemia.
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Hyperkalemia is common in ELBW infants (i.e., <1,000 g), especially in the first few
days after birth. Newborns are more resistant to cardiac arrhythmias secondary to
hyperkalemia than older children. Treatment is usually indicated when serum [K+] is >7
mEq/L. If acidosis is present, it should be corrected to increase transfer of potassium
from extracellular to the intracellular fluid compartment.
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6. Hypercalcemia is defined as total serum [Ca] >12 mg/dL or [ionized calcium] >1.5
mmol/L and is rare in newborns. Hypercalcemia may cause vomiting, hypotonia and
encephalopathy.
Causes of hypercalcemia include:
•Low serum phosphorus with bone demineralization
•Congenital hyperparathyroidism (primary or secondary)
• William’s syndrome
• Hypervitaminosis D
• Subcutaneous fat necrosis
• Adrenal insufficiency
• Thiazide diuretic therapy
• Hypophosphatasia
•Hyperthyroidism
•Blue diaper syndrome (abnormal tryptophan transport ).
Treatment of hypercalcemia:
•Correct underlying cause, if possible
•Adequate hydration
•Obtain consult with Endocrine Service
•Furosemide to increase calcium excretion
•Glucocorticoids to inhibit intestinal absorption of calcium and ↓ bone resorption
•Increase inorganic phosphate by giving oral phosphate solution (Neutra-Phos™
200 mg/mL) at a dose of 3-5 mg/kg. Avoid parenteral phosphate solution in
severely hypercalcemic infants.
7. Hypomagnesemia is unusual and is associated with persistent hypocalcemia.
Treatment is MgSO4 25-50 mg/kg/dose IV slowly over several minutes. This can be
repeated.
8. Hypermagnesemia, defined as a serum magnesium concentration >3 mg/dL, usually
occurs secondary to magnesium treatment of the mother as a tocolytic to stop preterm
labor or in mothers with pre-eclampsia. Neonatal symptoms include hypotonia,
hyporeflexia, hypotension and apnea, as well as vasodilatation with marked flushing.
The treatment is usually symptomatic until magnesium level gradually falls secondary to
renal excretion. With severe cases, administration of calcium IV (see above) may help.
Infants with severe hypermagnesemia may require assisted ventilation and blood pressure
support.
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Acid-Base Balance
INTRODUCTION: The newborn infant is subject to numerous conditions that may
disturb acid-base homeostasis. Management of ventilation, which controls the respiratory
component of acid-base balance, is discussed in the section on Respiratory Support (P.
10). This section is a brief discussion of the metabolic aspects of acid-base balance.
METABOLIC ACIDOSIS, defined as a base deficit >5 mEq/L on the first day and
>4 mEq/L thereafter, occurs from:
•Loss of buffer (mainly bicarbonate) or
•Excess production of acid or decreased excretion of acid
The anion gap is a useful calculation in assessing metabolic acidosis.
Anion gap = [Na+] – ([Cl-] + [HCO3-])
Loss of buffer has no effect on anion gap. Accumulation of organic acid (e.g., lactic
acid) causes an increase in anion gap.
Normal anion gap: <15 mEq/L
Increased anion gap:
>15 mEq/L in LBW infants (<2,500 g)
>18 mEq/L in ELBW infants (<1,000 g)
Newborn infants normally have a base deficit of 1 to 3 mEq/L.
Common causes of metabolic acidosis:
•Bicarbonate loss, especially via immature kidney or from GI tract
•Lactic acidosis from inadequate tissue perfusion and oxygenation (e.g., from asphyxia, shock,
severe anemia, hypoxemia, PDA, NEC, excessive ventilator pressures with ↓ cardiac output)
•Hypothermia
•Organic acidemia due to an inborn error of metabolism (see P. 155)
•Excessive Cl in IV fluids
•Renal failure
•Excessive acid load from high protein formula in preterm (late metabolic acidosis of
prematurity )
•Excretion of HCO3- as metabolic compensation for respiratory alkalosis
Dilution acidosis is caused by excessive volume expansion (with saline, Ringer’s lactate
or dextrose solutions). The extracellular space becomes “diluted” (relative decrease of
HCO3-); carbonic acid dissociates more and liberates more H+. Therefore, pH falls.
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Acid Base Balance
Loss of gastric fluid from vomiting or Replace deficit and give fluids and electrolytes
diarrhea with Cl- loss to keep pace with continuing losses.
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PREVALENCE: The rate of VLBW babies is increasing, due mainly to the increase in
prematurely-born multiple gestations, in part related to assisted reproductive techniques.
The distribution of LBW infants is shown in the Table:
________________________________________________________________________
RISK FACTORS: Any baby born prematurely is more likely to be very small. However,
other factors that can contribute to the risk of VLBW include:
•Race: African-American babies are twice as likely as Caucasian to be VLBW. Black
infants (16% of US live births) account for 37% of ELBW infants.
•Age: Teen mothers (especially if <15 years old) have a much higher risk of having
VLBW infant.
•Multiple birth babies are at increased risk of being VLBW because they often are
premature. More than 50% of twins and other multiple gestations are VLBW.
•Maternal health: Women exposed to drugs, alcohol, and cigarettes during pregnancy
are more likely to have LBW or VLBW babies. Mothers of lower socioeconomic
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VLBW Infants
status are also more likely to have poorer pregnancy nutrition, inadequate prenatal
care, and complications of pregnancy. All are factors that can contribute to VLBW.
NEONATAL COMPLICATIONS are markedly increased in VLBW, and especially
ELBW, infants. Because most VLBW infants are also premature, it may be difficult to
differentiate problems due to prematurity from those due to very small size. In general,
the lower a baby's birthweight, the greater are the risks for complications. However,
some complications of prematurity (e.g., risk of RDS) are lessened by the stress of mild
to moderate intrauterine growth restriction. Clinical problems associated with VLBW
and ELBW include:
1. Hypothermia: LBW infants have higher body surface area:body weight ratios,
decreased stores of brown fat and glycogen, and may not be able to conserve or
generate body heat. Clinical problems associated with hypothermia include
hypoglycemia, apnea, increased O2 consumption and metabolic acidosis. Prevention of
hypothermia increases survival of the infants. Methods of preventing heat loss include:
•Drying the infant at birth to prevent evaporative heat loss
•Warmed blankets or plastic wrap to prevent convective and radiant heat loss during
transport
•Swaddling to preserve body heat in larger infants, and radiant heater or a heated
incubator to maintain a neutral thermal environment for smaller infants.
2. Hypoglycemia due to decreased stores of glycogen and fat. Hypothermia and hypoxia
aggravate this due to increased metabolic demands and anaerobic glycolysis.
3. Perinatal asphyxia, especially among growth retarded infants because of
compromised O2 delivery in utero.
4. Respiratory problems:
•Respiratory Distress Syndrome, due to surfactant deficiency (see P. 79)
•Apnea of prematurity (see section on Apnea, P. 91)
5. Fluid and electrolyte imbalances due to increased insensible water loss (due to ↑
surface area/body weight, thin skin), impaired renal function. They are at risk for
dehydration, fluid overload, hypernatremia, hyponatremia, hyperkalemia (especially
ELBW), hypocalcemia, hypermagnesemia (iatrogenic from maternal treatment).
Compromised renal function may impair tolerance of free water, bicarbonate
resorption, potassium secretion, or urinary concentrating capacity.
6. Hyperbilirubinemia (see section on Jaundice, P. 118)
•Indirect (unconjugated) hyperbilirubinemia due to bruising or hemorrhage, ↓ RBC
survival, hepatic immaturity, delayed enteric feedings and ↓ gut motility. With
IUGR, risk factors may include infection and/or polycythemia.
•Direct (conjugated) hyperbilirubinemia as a complication of parenteral nutrition.
7. Anemia due to:
•Phlebotomy for laboratory tests and small total blood volume
•Anemia of prematurity
8. Impaired nutrition, feeding difficulties and slow rates of weight gain due to:
•Gut immaturity with decreased motility, enzyme deficiencies and ↑ risk of
necrotizing enterocolitis (see P. 133)
•Delayed enteric feeding due to respiratory disease, PDA, indomethacin treatment
•Infants <32-34 weeks gestation are developmentally not ready to nipple feed
•Increased caloric needs (↑surface area/body weight)
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13. Sudden infant death syndrome (SIDS): Premature infants are at increased risk, but
home monitoring has not been shown to be an effective preventive measure. Home
monitoring is not recommended in absence of other risk factors (e.g.. twin sibling with
SIDS, two siblings with SIDS, obstructive airway problems, or craniofacial anomalies
posing risks for obstructed airways).
MANAGEMENT: Because of the increased risk for multiple problems, these infants
require meticulous attention to all facets of their care. The following are but a brief
summary of certain aspects of the care of these fragile infants:
1. Resuscitation: (see section on Resuscitation, P. 1)
2. Respiratory Care: The majority of ELBW (i.e., <1,000 g) will require intubation
at birth (to assist in their cardiopulmonary adaptation to extra-uterine life) and assisted
ventilation for a prolonged period. They require close attention with frequent
measurements of pH and blood gas tensions. In addition to surfactant deficiency, they
are at risk for respiratory failure because of:
•Weak chest wall •Weak muscles of respiration
•Smaller alveoli (↑ tendency to atelectasis) •Decreased central respiratory drive
3. Cardiovascular: Most VLBW and almost all ELBW infants will require an umbilical
arterial catheter for blood sampling and blood pressure measurement. Hypotension is
common. The most effective therapy is dopamine (usual starting dose is 5
mcg/kg/min). Do not automatically give fluid boluses for “decreased perfusion,”
acidosis, or hypotension. Excess fluid will worsen pulmonary function and give excess
Na+. Reserve volume expansion for situations where there are signs of hypovolemia
(see sections on Shock, P. 101, and Blood Pressures, P. 35).
3. Oxygen therapy: Maintain SpO2 in range of 85-92%. If SpO2 is > 94%, arterial
oxygen tension may be high (>100 mmHg) because of the inaccuracy of the pulse
oximeter at high saturations. This puts the infants at ↑ risk for ROP. Do not write
titration orders for oxygen.
4. Fluids: On the 1st day of life, preterm infants should receive restricted fluids (e.g., 60-
80 mL/kg/d). However, for ELBW infants, fluid intake should be higher (e.g., 100-
125 mL/kg/d. Follow intake and output closely, at least q12h for the first several days.
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5. Electrolytes: On the 1st day, do not give Na+ or K+. To avoid hypocalcemia, start Ca
gluconate at 200 mg/kg/d. Follow serum electrolytes closely.
6. Nutrition: Feedings on the 1st day of life are unusual for VLBW infants. Do not start
feeds on the 1st day of life in ELBW infants. (see section on Feeding, P. 50). Trophic
(gut stimulation) feedings for several days facilitate later advance of feedings.
Consider early institution of TPN. Do not give IV lipids for 3-5 d, especially if there
is severe pulmonary disease. (see section on Parenteral Nutrition, P. 136).
7. Infection: Obtain CBC and blood culture at birth. If there are any risk factors, begin
antibiotic therapy (48 h of treatment until culture results are known).
8. Glucose: Maintain blood glucose ≥45 mg/dL. (see section on Hypoglycemia, P. 153).
Initial IV fluid should be D10W. Some ELBW infants may become hyperglycemic and
require lower glucose intake and/or insulin.
9. Hyperbilirubinemia: (see section on Jaundice, P. 118)
10. Anemia: Assume all ELBW and many VLBW infants will need at least 1 transfusion.
Obtain parental consent in advance, discussing option for designated donor blood. Type
and cross match packed cells in small volume aliquots to minimize number of donors.
Start erythropoietin as described in Guidelines for Use of Erythropoietin (P. 107).
11. Intraventricular hemorrhage (see schedule for cranial sonograms on P. 146)
12. Ophthalmology examination for ROP commencing at age 1 mo for infants born <
32 weeks.
OUTCOME:
Survival of VLBW babies is directly related to birth weight. Survival data for infants
born at UCSF from 1998-2002 (inclusive) are:
Birth Weight (g) Survival
500-750 74%
751-1,000 82%
1,001-1,250 92%
1,251-1,500 95%
Long-term outcome: VLBW and ELBW infants are at ↑ risk for cerebral palsy,
developmental delay, mental retardation, visual problems (including blindness), hearing
impairment, chronic lung disease and SIDS. Risk for these ↑ with decreasing BW and
GA. Data for very preterm infants followed in the UCSF ICN Follow-Up Clinic are
shown below:
GA* No Deficits‡ One Deficit‡ Two or More‡
24 28% 39% 33%
25 47% 23% 30%
26 63% 34% 3%
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Intrauterine Growth Retardation
B. Uterine and placental factors that can adversely affect fetal growth include
inadequate placental growth, uterine malformations, decreased utero-placental blood
flow (e.g., toxemias of pregnancy, diabetic vasculopathy) and multiple gestations
C. Fetal causes are unusual, include familial genetic and chromosomal abnormalities and
intrauterine infections (i.e., TORCH), and usually have a poor long term prognosis.
PATHOPHYSIOLOGY: With maternal or placental causes of IUGR, there is decreased
placental transfer of nutrient (including oxygen) resulting in reduced fetal body stores of
lipids and glycogen resulting in neonatal hypoglycemia; chronic hypoxemia stimulates
erythropoietin production leading to polycythemia. These infants are also at increased
risk for perinatal asphyxia. Other associated problems include hypocalcemia, pulmonary
hemorrhage, hypothermia and, with IUGR associated with toxemia, thrombocytopenia
and leukopenia. With fetal causes, decreased growth is constitutive (due to genetic
factors) or secondary to infection.
ASSESSMENT and MANAGEMENT:
-Treat asphyxia if present.
-Measure weight, head circumference and length to categorize the type of IUGR.
-Careful physical examination for anomalies and dysmorphic features.
-Blood glucose and hematocrit to detect hypoglycemia and polycythemia. See
sections on Hypoglycemia (P. 153) and on Polycythemia (P. 112).
-Serum Ca++, WBC count with differential and platelet count.
-Infants with IUGR due to placental factors have ↑ O2 consumption. This ↑ insensible
water loss to a variable degree (as much as 20-30%). Compensate for this by
increasing IV fluid intake. These infants may also need greater intake (>150 mL/kg/d
and >100 kcal/kg/d) to achieve adequate growth.
-Further workup and treatment depends on abnormalities identified on history and
physical examination.
OUTCOME:
-Perinatal mortality for IUGR infants is 5-20 times greater than for AGA, mainly due
to intrauterine death, perinatal asphyxia, and congenital anomalies.
-Neurologic morbidity is 5-10 times higher than for AGA infants, especially for
infants with ↓ head circumference at birth. Intellectual and motor function (excluding
those with congenital infections, chromosomal abnormalities) depends on adverse
perinatal events and on the specific cause of growth restriction. Early identification
and treatment of hypoglycemia and polycythemia improves outcome. Neurologic
abnormalities are usual with genetic and infectious causes of IUGR.
-Retarded growth: With placental causes of IUGR, catch-up growth occurs after birth,
but these patients usually remain smaller than expected.
-Fetal “programming” of cardiovascular disease: Recent studies implicate IUGR
with adult onset of hypertension, coronary heart disease, hypercholesterolemia, and
diabetes. These studies suggest that IUGR has long term affects on endocrine
development and homeostasis.
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Immunizations
• If a decision is made to never immunize an infant (e.g., if the parents do not want their infant
immunized), the infant’s Nurse will document this on the immunization record in the medical
record and the Pharmacy will not include this patient on future notification lists.
•Unless contraindicated, all patients should receive Acetaminophen (10 mg/kg PO) 1-2h prior to
DTaP immunization and q4-6h after for 24 hours.
IMMUNIZATION SCHEDULE for ICN:
Mother’s HbsAg Status
Age Negative Positive Unknown
Birth HBV #1 (by age 12 h) HBV #1 (by age 12 h)
HBIG “ HBIG*
*Determine maternal HbsAg status after birth; if positive, give HBIG by age
7d for full term infants; for preterm, give HBIG within 12h after birth..
1 month HBV#2
2 months IPV #1 IPV #1
DTaP #1 DTaP #1
COMVAX #1 HIB #1
PCV7 #1 PCV7 #1
4 months IPV #2 IPV #2
DTaP #2 DTaP #2
COMVAX #2 HIB #2
PCV7 #2 PCV7 #2
6 months‡ DTaP #3 DTaP #3
PCV7 #3 PCV7 #3
HIB #3
HBV #3
‡
Consider giving Influenza vaccine to infants with chronic lung disease or cardiac disease.
12-15 months IPV #3 IPV #3
COMVAX #3
Discharge Planning
INTRODUCTION: Preparing an ICN patient for discharge involves several individuals
and careful planning. For most infants, discharge planning can begin at admission when
the physician formulates the treatment plan. Below are guidelines to facilitate the
discharge process, avoid delays and ensure continuing care after the baby leaves UCSF.
SOCIAL SERVICE ROUNDS are held weekly at 11:00 AM on Wednesday. Each
patient is discussed by the medical team, Social Workers and Discharge Coordinators.
Current patient care plans are discussed and special problems may be identified that will
affect discharge. These may include adequacy of the parents, the home and available
resources given the medical needs of the infant, availability of necessary follow-up care,
transport to home or to a hospital nearer to home, and special medications, supplies or
devices that the infant will need.
CRITERIA FOR DISCHARGE: For growing preterm infants, discharge can be
anticipated when the infant:
•weighs ≥1,800 g
•is gaining weight steadily on nipple feedings (breast or bottle)
•can maintain body temperature in an open crib
•has had no episodes of apnea for at least 5d
•has an adequate home environment
Criteria for infants with other medical/surgical conditions vary with the clinical situation.
HELPFUL TIPS FOR PLANNING AN INFANT’S DISCHARGE:
•Keep “Bumble-Bee” (discharge) form up to date during the infant’s hospitalization.
•Check with the parents to determine who will be the primary physician after
discharge.
•Stabilize the discharge medications at least 3d before discharge.
•For infants on multiple medications, cluster the dose times to minimize the number of
times the parents have to give medications.
•For preterm infants on formula feedings, monitor weight gain, on caloric density that
the infant will be receiving at the time of discharge, for at least 3d before discharge.
•The discharge physical examination can be done up to 24h before discharge.
•Confirm the discharge time and follow-up plans with all consulting teams.
•Check with infant’s Social Worker regarding any special family needs or problems.
•Notify the Discharge Coordinator at least 3 days before the planned discharge time.
•Notify parents at least 3d before the planned discharge. They may have several
questions, be very anxious and need time to adjust to the idea of taking home their
infant, who has been so sick.
•For non-English speaking parents, arrange for a translator to be present at the
discharge conference.
•If the infant is to be transferred to another hospital, give at least 24h notice to the
Attending or Fellow so the Discharge Summary can be completed on time.
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Well Baby Nursery
Pedi-Med Service
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Well Baby Nursery
Pedi-Med Service
SEPSIS, HIV, AND HEPATITIS: Please refer to the sections on infectious diseases in
this manual.
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Well Baby Nursery
Pedi-Med Service
POLICE HOLD POLICY: A Police Hold is a process whereby the police place an
infant or minor child in temporary protective custody of the Juvenile Court. It removes
physical custody from the parents or guardians, and prevents the family from removing
the child from the hospital without the express permission of Child Protective Services.
While on a police hold, parents retain legal custody and may continue to consent to
treatment and procedures. To initiate a police hold, contact the UCSF Police Department.
Circumstances which necessitate a police hold include:
•A parent threatens to remove a baby against medical advice, and the discharge is
medically unsound and/or life threatening.
•A parent is violent or is threatening violence.
•A parent is incapacitated by drugs or alcohol and the baby’s immediate safety is in
question.
•A parent is incapacitated by mental illness and the baby’s immediate safety is in
question.
Parents must be informed of the hold by the medical staff. A police hold does not
necessarily prevent parents from visiting and participating in their infant’s care.
However the hospital has the right to set limits on visitation to ensure patient, staff and
visitor safety. Nursing will ensure that all visits are supervised.
The entire Police Hold Policy can be found in the Well Baby Nursery Manual.
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Respiratory Distress Syndrome
subsequent lactic acidosis. Hypoxemia and acidosis may further impair oxygenation by
causing pulmonary vasoconstriction, resulting in right-to-left shunting at the levels of the
foramen ovale and ductus arteriosus.
Other factors, such as PREMATURITY
baro/volutrauma and high FIO2, may
Surfactant Structurally
initiate release of inflammatory Deficiency Atelactasis Immature Lung
cytokines and chemokines causing
more endothelial and epithelial cell V/Q Mismatch Hypoventilation
injury. The injury results in reduced
surfactant synthesis and function as ACUTE
Hypoxemia & Hypercarbia
CHRONIC
surfactant inactivation.
Impaired endothelial and Cytokine Free-radical
Macroscopically, the lungs appear epithelial integrity Release reactions
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Respiratory Distress Syndrome
•Optimize fluid management: avoid fluid overload and resultant body and pulmonary
edema while averting hypovolemia and hypotension
•Reduce metabolic demands and maximize nutrition
•Minimize lung injury secondary due to volutrauma and oxygen toxicity
The three most important advances in prevention and treatment of RDS have been: (a)
antenatal glucocorticoids, (b) continuous positive airway pressure (CPAP) and positive
end-expiratory pressure (PEEP), and (c) surfactant replacement therapy. These have
dramatically decreased morbidity and mortality from RDS.
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Respiratory Distress Syndrome
with the advantages of avoiding mechanical ventilation and volutrauma. The current
approach to the timing of surfactant therapy at UCSF is summarized in Table 3.
______________________________________________________________________
Table 3. Guidelines for intubation and timing of surfactant administration in
preterm infants.
Gest. Age Antenatal Ventilatory Timing of
(Weeks) Steroids* Treatment Surfactant
≤27 No Intubate all Prophylactic
infants
≤27 Yes (a) If intubation† & mech. Prophylactic, unless
vent. needed at birth in room air by age 20 min
(b) Early CPAP, if not stable Rescue
intubate† & mech. vent.
(c) Early CPAP, if stable Do not give surfactant
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Respiratory Distress Syndrome
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Respiratory Distress Syndrome
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Pulmonary Hypoplasia and
Congenital Diaphragmatic Hernia
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CLINICAL PRESENTATION:
-Term or post-term infant (In preterm infants, the pulmonary vasculature is rarely
sufficiently developed to result in PPHN.)
-Onset at birth or within a few hours
-History or clinical findings consistent with condition associated with PPHN
-Cyanosis, often with pre-ductal (right upper extremity) O2 saturation >post-ductal
-Respiratory distress is common
-Chest radiograph clear (idiopathic PPHN) or abnormal due to associated condition
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Persistent Pulmonary Hypertension
As patient improves with treatment, wean oxygen and ventilatory support slowly.
Frequently in infants with PPHN, oxygenation will decrease suddenly and dramatically
after small decreases in FIO2 or ventilator pressures, or with airway suctioning, and it
may take several hours for oxygenation to recover.
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Because of the risk of rebound pulmonary hypertension, be certain that the bag system
(for manual ventilation) is set up to deliver iNO at the time of the onset of iNO therapy.
This will ensure that the infant will continue to receive iNO during suctioning of the
airway and in the event of a malfunction of the ventilator.
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Pulmonary Hemorrhage
DEFINITION: Pulmonary hemorrhage (P-Hem) is an acute, catastrophic event
characterized by discharge of bloody fluid from the upper respiratory tract or the
endotracheal tube. The incidence of P-Hem is 1 in 1,000 live births. P-Hem is present in
7 to 10% of neonatal autopsies, but up to 80% of autopsies of very preterm infants.
When evident clinically, P-Hem is usually massive, is associated with bleeding in other
sites, involves more than one third of the lungs, and has a high mortality rate.
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DIFFERENTIAL DIAGNOSIS:
1. Apnea of prematurity, most common in infants ≤34 weeks gestation. (Note: All
following etiologies must be excluded or appropriately treated before a diagnosis of
apnea of prematurity can be made).
2. Hypoxemia
3. Infection (sepsis, meningitis, pneumonia)
4. Necrotizing enterocolitis
5. Intracranial hemorrhage
6. Hydrocephalus
7. Seizures
8. Patent ductus arteriosus
9. Hypoglycemia
10. Anemia (Note: Apnea may improve after PRBC transfusion; however, infants who
respond cannot be predicted by their Hct. Infants with a low Hct who respond to
transfusion usually have an elevated blood lactate and elevated heart rate.)
11. Polycythemia/hyperviscosity
12. Atelectasis
13. Gastroesophageal reflux. Methylxanthines may exacerbate reflux. If reflux
associated with apnea becomes a persistent problem, the head of the bed should be
elevated 20 degrees. Metoclopramide (Reglan™: 0.1 – 0.2 mg/kg/dose, TID or
QID), bethanechol, and H2 blocking agents should be used only after reflux has
been documented with pH probe analysis.
14. Feeding bradycardia (probably due to vagal stimulation from the NG tube or from
gastric distension)
15. Following anesthesia or depressant drugs (This is frequently observed after repair
of inguinal hernias in former preterm infants and can be minimized with
perioperative caffeine administration. The susceptibility of preterm infants to apnea
after anesthesia may persist until 50-60 post conceptional weeks.)
16. Maternal drug withdrawal
17. Elevated environmental temperature
18. Upper Airway obstruction (e.g., due to nasal secretions, choanal atresia or stenosis,
vocal cord paralysis)
19. Tracheal suctioning (due to hypoxemia and/or vagal stimulation)
20. PGE1 infusion
21. Congenital Hypoventilation Syndrome (Methylxanthines are ineffective and these
infants need mechanical ventilation.)
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Apnea and Bradycardia
TREATMENT: If the infant has any of the above conditions, these should be treated
appropriately.
- Monitoring: Infants at risk for apnea should have continuous monitoring of respiration
and heart rate (because an apnea monitor alone will miss obstructive apnea) until they
are at least 34 wks postconceptional age and have been free of apnea and bradycardia for
one week.
-Prevent hypoxemic episodes.
-Cutaneus stimulation is effective with mild apnea.
-Maintain the infant in prone position.
-Methylxanthines: When idiopathic apnea of prematurity is severe enough to treat
pharmacologically, the drug of choice is p.o. caffeine (even when the child is n.p.o. –
except during necrotizing enterocolitis). A loading dose of caffeine citrate 20 mg/kg
p.o. (10 mg/kg caffeine base) should be used, followed in 24 h by a daily maintenance
dose of 5.0 mg/kg/d caffeine citrate. Therapeutic levels will usually be achieved within
30–120 minutes of the initial dose. Plasma caffeine levels are usually not measured,
because some infants may require higher plasma levels for response and because
caffeine toxicity is not generally a problem until plasma levels >50 mg/L. For infants
who fail to respond to the above dosages, a second load of 10–20 mg/kg caffeine citrate
can be given, followed by a maintenance dose of 7.5 mg/kg/d. If symptoms that could
be attributable to methylxanthine toxicity occur (most commonly tachycardia, jitteriness
and vomiting), theophylline concentrations as well as caffeine concentrations should be
measured. Because of the long half-life of caffeine, therapeutic levels may persist for
>7–14 d after discontinuing therapy. Caffeine therapy needs to be discontinued well
before discharge.
-Respiratory support: Continuous air flow through nasal cannulae is useful in some
infants, and others will respond to nasal CPAP. If apnea is severe, the infant may
require mechanical ventilation.
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Chronic Lung Disease
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Congenital Heart Disease
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Congenital Heart Disease
3. Cyanosis with ↓ pulmonary blood flow: The most common causes are right-sided
obstructive lesions that result in inadequate blood flow to the lungs. Age of
presentation varies depending on the lesion. Whereas tricuspid atresia presents rapidly
immediately after birth, mild forms of Ebstein’s anomaly may not present until later in
life. With some lesions, cyanosis decreases as pulmonary vascular resistance drops.
A. Likely lesions include:
•Tetralogy of Fallot (with or without pulmonic atresia). In mild cases, cyanosis
may not be present at birth (acyanotic Fallot, “pink tet”).
•Tricuspid atresia, pulmonic atresia with intact ventricular septum, severe
pulmonic stenosis
•Ebstein’s anomaly of the tricuspid valve
B. Management: In most cases, infusion of PGE1 increases pulmonary blood flow
and stabilizes the infant until surgical intervention is undertaken. Assisted
ventilation and increased environmental oxygen are often necessary. For these
infants, oxygen saturations in the 75-85% range are adequate.
4. Shock: The differential diagnosis of shock is discussed in the section on Shock (P.
101). The most common types of CHD that cause shock are left-sided obstructive
lesions. The mechanism of shock is inadequate systemic blood flow. The onset of
signs relates to closure of the ductus arteriosus which may not occur until a few days
after birth.
A. Likely lesions include:
•TAPVR with obstruction may present with both respiratory distress and shock.
•Mitral atresia or severe mitral stenosis (type of hypoplastic left heart complex)
•Aortic atresia or severe aortic stenosis (type of hypoplastic left heart complex)
•Interrupted aortic arch (often associated with DiGeorge Syndrome)
•Coarctation of aorta (usually presents several days after birth)
B. Management:
•PGE1 to allow right→left shunting through ductus arteriosus
•Assisted ventilation to treat pulmonary edema (as well as apnea from PGE1)
•Correct metabolic acidosis (see sections on Resuscitation, P. 1, and Acid Base
Balance, P. 62)
•Inotropic agents to improve myocardial function
•Be cautious with use of oxygen in setting of a single ventricle. Discuss use of
oxygen with Fellow or Attending. Oxygen will accelerate the closure of the
ductus arteriosus and worsen infant’s condition by decreasing systemic
output. Therefore, do not increase environmental oxygen until after PGE1
has been started.
•Fluid restriction if there is renal failure
•In some cases of hypoplastic left heart, there is excessive pulmonary blood flow
and inadequate systemic output, even with PGE1. In such cases, the following
measures may be used in an attempt to decrease pulmonary blood flow and
increase systemic output. These infants require mechanical ventilation and it is
usually necessary to administer muscle relaxants (i.e., pancuronium bromide) to
prevent the infant from hyperventilating:
-Decrease inspired oxygen to ~18% by adding N2 to inspired gas (to ↓
alveolar PO2)
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Congenital Heart Disease
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MEDICAL TREATMENT:
General Considerations: Conventional treatment of congestive heart failure (fluid
restriction, diuretics, digoxin) is not effective and will delay proper treatment. Unless the
PDA is not hemodynamically significant, infants with PDA should be NPO.
Indomethacin:
1. Gestation ≥28 weeks at birth: These infants are usually treated only when a
hemodynamically significant PDA is present. This practice is aimed to avoid unnecessary
treatment. A PDA is considered hemodynamically significant if, in addition to a murmur,
two or more of the following signs are present:
-increased pulse volume or widened pulse pressure
-hyperactive precordium
-increased pulmonary vascular markings on chest radiograph
An untreated hemodynamically significant PDA will prolong the need for oxygen therapy
and delay the establishment of feedings. An echocardiogram should be obtained to rule
out structural congenital heart disease.
Indomethacin Dosage (IV):
Birthweight >1,250 g: Give three doses of 0.2 mg/kg; give the 2nd dose 12 h after the
first, and the 3rd dose 24 h after the 2nd.
Birth weight 1,000-1,250 g: 1st dose is 0.2 mg/kg; 12 h later, give 0.1 mg/kg for 2nd
dose; 24 h after the 2nd dose, give 0.1 mg/kg.
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Patent Ductus Arteriosus
Contraindications to Indomethacin:
-Active bleeding:GI and other (Note that presence of an ICH is not a contraindication)
-Active or suspected Necrotizing Enterocolitis (NEC)
-Creatinine ≥2.0 mg/dL
-Urine output <1 mL/kg/h (indomethacin may be restarted when urine output
>1mL/kg/h)
-Platelet count <50,000 (consider platelet transfusion prior to indomethacin)
-Active (and untreated) infection
-Suspected Congenital Heart Disease
-Known gastrointestinal or renal anomaly
Management During Indomethacin Treatment: Because indomethacin decreases
gastrointestinal blood flow, infants should be kept NPO until at least 48 h after the
indomethacin therapy has been completed.
Because indomethacin may cause a transient decrease of urine output (similar to
excessive ADH action), IV fluids should be adjusted every 8-24 h, taking into
consideration not only fluid intake in mL/kg, but more importantly, the relationship
between urine output and fluid intake. An acceptable output to intake ratio in these
circumstances is between 0.3 and 0.7, taking the infant’s anticipated weight change into
consideration. A decrease in serum Na+ should prompt additional fluid restriction
because of retention of free water with indomethacin administration.
SURGICAL CLOSURE OF PDA should be considered in the following situations:
-Failure of indomethacin therapy
-Hemodynamically significant PDA and presence of contraindication(s) to
indomethacin
-Presence of PDA and NEC. In this circumstance, operative closure of the PDA is
almost always necessary before the NEC will resolve and may be required as an
emergency procedure.
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Neonatal Shock
DEFINITION AND PATHOPHYSIOLOGY: Shock is an acute state in which
circulatory function is inadequate to supply sufficient amounts of O2 and other nutrients
to tissues to meet metabolic demands. In most cases, cardiac output is low. In early
shock, compensatory regional vasoconstriction (skin, skeletal muscle, splanchnic
circulation) may temporarily maintain normal blood pressure and adequate blood flow to
vital organs. As shock progresses, compensatory mechanisms fail and widespread
cellular damage occurs. Insufficient O2 delivery to tissues causes anaerobic metabolism
and lactic acidosis. If shock persists, irreversible injury to vital organs occurs; death
ensues despite vigorous therapy that may temporarily return cardiovascular
measurements to normal.
ETIOLOGY: A variety of factors, often in combination, may cause shock:
Hypovolemia (e.g., blood loss, inadequate placental transfusion, feto-maternal
transfusion, severe dehydration)
Asphyxia (e.g., antepartum or intrapartum, respiratory failure, impaired oxygen
transport due to severe anemia or hemoglobinopathy)
Cardiogenic causes (e.g., cardiomyopathy, dysrhythmia, congenital malformation,
hypocalcemia, severe hypoglycemia)
Obstruction of venous return due to tension pneumothorax, excessive
ventilator pressures, cardiac tamponade
Sepsis (especially early onset group B beta-hemolytic Streptococcal infection)
Drugs (especially when hypovolemic infants, in whom blood pressure has been
maintained by vasoconstriction, are given vasodilators such as PGE1,
isoproterenol or magnesium)
Hypocarbia (severe)
CLINICAL EVALUATION: There are no clinical or laboratory findings specific to
shock. The diagnosis is based on presence of several indicators of inadequate circulatory
function. Various clinical and laboratory findings associated with shock include:
Cardiovascular findings:
Systemic arterial hypotension (see graphs on P. 36)
Narrow pulse pressure
Central venous hypotension: with myocardial failure, central venous pressure is ↑
Tachycardia (In early asphyxia, bradycardia is present.)
Respiratory findings:
Tachypnea Grunting respirations
Retractions Apnea
Other findings:
Prolonged capillary filling time Hypothermia
Oliguria Metabolic Acidemia
TREATMENT: Specific therapy depends upon the cause of shock. Rapid recognition of
shock and identification of underlying cause(s) are essential to prevent irreversible
changes. With hypovolemia, intravascular volume must be increased, but cautiously.
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Neonatal Hypertension
DEFINITION: Systolic or mean arterial blood pressure (BP) >95th percentile for birth
weight, gestational age and post-natal age. (See graphs of normal BP on P. 36.)
EPIDEMIOLOGY:
-95th percentile for systolic BP = 65 mmHg at 24 weeks and 90 mmHg at 40 weeks post-
conception.
-By definition, 5% of all infants have a blood pressure above the 95th percentile, but real
incidence of hypertension in ICN is about 0.8%
-In infants with bronchopulmonary dysplasia (BPD), intraventricular hemorrhage (IVH),
umbilical arterial catheters (maintained in “high” position) or patent ductus arteriosus,
incidence of hypertension is 9%.
-Incidence varies widely in different ICNs.
SIGNS/CONSEQUENCES include lethargy, irritability, apnea, tachypnea, seizures,
intracranial hemorrhage, congestive heart failure or cardiogenic shock.
DIFFERENTIAL DIAGNOSIS:
-Commonest cause of neonatal hypertension is reno-vascular hypertension due to
thrombi from “high” umbilical arterial catheter (UAC). Maintain UAC so that tip is
below 3rd lumbar vertebra (L3). (See section on Catheters, P. 25)
-Other renal causes: thromboembolism, renal artery stenosis, coarctation of aorta, renal
vein thrombosis, renal anomalies, polycystic or dysplastic kidneys, acute tubular
necrosis
-Iatrogenic: glucocorticoids, dopamine (and other pressors), caffeine, pain, fluid/Na+
disturbances
-Neurological: intracranial hypertension, seizures, IVH, subdural hematoma
-Endocrine: congenital adrenal hyperplasia, hyperthyroidism, hyperaldosteronism
-Pulmonary: hypercarbia, chronic lung disease, pneumothorax (early, followed by
hypotension)
EVALUATION:
-Document presence of hypertension with multiple limb BPs and invasive measurement,
if possible.
-History: recent and current medications, procedures (UAC)
-Physical examination, including state (pain, agitated, seizure, quiet, etc.)
-Laboratory: BUN, Creatinine, electrolytes, Ca, UA with micro, chest radiograph, renal
ultrasound with Doppler flow studies, cranial ultrasound
-Other laboratory tests (in selected infants with consultation): thyroid function tests,
urinary VMA/HVA, cortisol, aldosterone, echocardiography
-Further workup and management with appropriate consultation (e.g., nephrologist,
cardiologist, endocrinologist)
TREATMENT varies with cause of hypertension. Treat the etiology, if possible.
Correct or treat iatrogenic causes!
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Tachyarrhythmia Bradyarrhythmia
narrow-QRS wide-QRS
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Neonatal Cardiac Arrhythmias
I. Reentry tachycardias
Diagnosis Findings on ECG Treatment
Atrial flutter -"Sawtooth" flutter waves -Unstable: esophageal pacing or
-AV block does not terminate atrial rhythm electrical cardioversion
-Atrial rate up to 500 in newborns
-Variable AV conduction common -Stable: digoxin, propranolol,
or digoxin + procainamide
Accessory pathway mediated -P follows QRS, typically on upstroke of T
tachycardia (WPW) -Superior or rightward P wave axis
-AV block always terminates tachycardia -Unstable: esophageal pacing or
-Typically terminates with P wave electrical cardioversion
-After termination, WPW have pre-excitation
-Stable: vagal maneuvers.
Permanent form of junctional -Incessant - P wave precedes QRS adenosine
reciprocating tachycardia (PJRT) -Inverted P waves in II, III, AVF propranolol or digoxin
-AV block always terminates tachycardia
-May terminate with QRS or P wave -No response: procainamide or
-No pre-excitation after termination flecainide
Atrioventricular node reentry -P usually not visible, superimposed on QRS
-AV block usually terminates tachycardia.
Atrial and sinoatrial reentry -P present, precedes next QRS -Unstable: electrical cardioversion
-Terminates with QRS rather than P -Stable: propranolol, procainamide
-AV block does not terminate atrial rhythm or amiodarone
-P axis may be superior or inferior
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C. Bradyarrhythmias:
Sinus bradycardia -Slow atrial rate with normal P waves -Vigorous resuscitation and
-1:1 conduction supportive care
-Due to underlying causes such as hypoxia, -A B C
acidosis, increased intracranial pressure, -O2
abdominal distension, hypoglycemia, -Treat underlying causes
hypothermia, digoxin, propranolol
Atrioventricular block -Atrioventricular dissociation -Unstable: A B C
-Regular R-R intervals O2
Complete atrioventricular block -Regular P-P intervals Atropine, isoproterenol
-Atrial rate > ventricular rate infusion
-P which occur after T have no effect on Temporary trans-
R-R interval venous pacing
-Infants of maternal lupus -Stable: Treat underlying causes
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Neonatal Anemia
BACKGROUND and PATHOPHYSIOLOGY: Normal erythropoiesis is influenced by
several factors, especially erythropoietin (EPO), which stimulates maturation of red blood
cell (RBC) precursors. Anemia, defined as hematocrit (Hct) or hemoglobin (Hgb)
concentration >2 SD below mean for age, may be due to three general causes: blood loss,
↑ RBC destruction or ↓ RBC production. The major physiologic impact of anemia is ↓
oxygen delivery to tissue, resulting in both compensatory responses (see “symptoms”)
and acute or chronic consequences including poor growth, decreased activity and limited
cardiovascular reserve. Anemia is defined as Hct <45% in a term infant. The table at the
end of this section gives some normal hematological values for preterm and term
newborn infants.
CAUSES OF NEONATAL ANEMIA:
1. Blood loss, the commonest cause of neonatal anemia, including:
A. Obstetrical causes: placental abruption, placenta previa, trauma to placenta or
umbilical cord during delivery and rupture of anomalous placental vessels
B. Feto-maternal transfusion: 8% of normal pregnancies have some admixture.
C. Feto-placental transfusion due to positioning of infant above level of placenta
after delivery, partial cord occlusion (e.g., with nuchal or prolapsed cord).
D. Twin-twin transfusion(see section on Multiple Births, P. 170):
•Occurs only with monochorionic (i.e., monozygotic) twins and when there are
placental vessels which allow shunting of blood from one twin to the other.
•Donor will have anemia of variable severity.
•Recipient will have polycythemia of variable severity.
E. Internal hemorrhage such as intracranial hemorrhage, subgaleal hemorrhage,
cephalohematoma, adrenal hemorrhage, subcapsular hematoma of liver or
ruptured viscus
F. Iatrogenic blood loss secondary to sampling of blood for laboratory tests. This is
the commonest cause of anemia (and transfusion) in small preterm infants.
2. ↑ RBC destruction:
A. Intrinsic causes: Hereditary RBC disorders (rare), including:
•RBC Enzyme defects (e.g., G6PD deficiency)
•RBC membrane defects (e.g., hereditary spherocytosis)
•Hemoglobinopathies (e.g., α-thalassemia)
B. Extrinsic causes:
•Immune hemolysis
-Rh incompatibility
-ABO incompitability
-Minor blood group incompatibility (e.g., Kell, Duffy)
-Hemangiomas (Kasabach Merritt syndrome)
•Acquired hemolysis:
-Infection
-Vitamin E deficiency (of historical interest, now it is very rare)
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-Drugs
3. ↓ RBC production:
A. Anemia of prematurity due to transient deficiency of erythropoietin
B. Aplastic or hypoplastic anemia (e.g., Diamond-Blackfan)
C. Bone marrow suppression (e.g., with Rubella or Parvovirus B19 infection)
D. Nutritional anemia (e.g., iron deficiency), usually after neonatal period
CLINICAL FINDINGS vary with the severity of anemia and other associated
conditions. There may be no signs with mild anemia. With more severe anemia, findings
include:
•Pallor •Tachycardia
•Tachypnea •Apnea
•↑ O2 requirements •Lethargy
•Poor feeding •Hepatosplenomegaly (hemolytic disease)
•Jaundice •Wide pulse pressure
•Hypotension •Metabolic acidosis with severe anemia
•↓ tolerance of labor with fetal anemia
DIAGNOSTIC EVALUATION of anemia:
1. History:
•Family: Anemia, ethnicity, jaundice
•Maternal and perinatal: Blood type and Rh; anemia; complications of labor or
delivery
•Neonatal: Age of onset; presence of other physical findings
2. Laboratory Evaluation may include the following, depending history and physical
findings:
•CBC with platelets, smear and reticulocyte count
•Blood group and type, Direct Antiglobulin test (Coombs Test)
•Bilirubin (total and direct)
•Kleihauer-Betke Test (on maternal blood to look for fetal red cells as evidence of
feto-maternal hemorrhage)
•Ultrasonogram for internal bleeding (head, abdomen)
•Rarely, hemoglobin electrophoresis and RBC enzymes
•Bone marrow aspiration is almost never necessary to diagnose anemia in a newborn
MANAGEMENT will depend on cause and severity of anemia.
1. Prenatal: Diagnosis of significant fetal anemia is unusual except in hemolytic disease
of the newborn (see P. 121) and Parvovirus B-19 infection. Fetal transfusion may be
needed for severe anemia.
2. Postnatal:
A. Anemia of prematurity: The main methods of management are:
•Limit blood drawing for laboratory tests
•Treatment with recombinant human erythropoietin (r-Hu-EPO) (see Guidelines
for Use of Erythropoietin, P. 111)
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•Transfusion with packed red blood cells (PRBCs) for severe anemia (see
guidelines on P. 40-41)
B. Other causes of anemia: Treat underlying cause when feasible. Transfusion
guidelines for treatment of anemia in newborns are given in the section, Administration
of Blood Products (P. 40).
C. Severe anemia: With severe, symptomatic anemia, the infant’s cardiovascular
system may not be able to tolerate the ↑ blood volume from simple transfusion of
PRBCs. In such cases, perform a partial exchange transfusion with PRBCs. See
sections on Exchange Transfusion (P. 42) and Polycythemia (P. 112) for the technique.
To calculate the volume of PRBCs to exchange, use the following formula:
Gestation
(weeks) Hct (%) Hgb (g/dL) Retic (%)
32 47 15.0 3-10
28 45 14.5 5-10
26-30 41 13.4 __
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IRON: Start oral iron at 2 mg/kg/d as soon as tolerated and increase to 4 mg/kg/d when
feeds reach 100 mL/kg. If not on iron after 2 weeks of r-Hu-EPO treatment, consult with
Dr. Shannon or Dr. Phibbs.
Consider:
1. Intravenous iron (1 mg/kg/d in intravenous alimentation fluid) or
2. Discontinue r-Hu-EPO.
When at full feeds, start UCSF preterm vitamins (see section on Vitamins, P. 55).
POST THERAPY: Hct and reticulocyte count will decline. Endogenous EPO will be
released only when the infant becomes anemic (usually at Hct in the mid 20s). Only then
will reticulocytes rise again. If reticulocyte count has not started to rise at the time of the
infant’s discharge, alert the primary MD to the need to follow the Hct and reticulocyte
count.
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Polycythemia/Hyperviscosity
INTRODUCTION: Polycythemia, defined as a venous hematocrit (Hct) >65%, occurs
in about 4% of term infants, and is more common with SGA (IUGR), LGA, infants of
diabetic mothers, trisomy 21, twin-twin transfusion, Beckwith-Wiedemann syndrome and
otherwise normal infants who receive an excessive placental transfusion.
PATHOPHYSIOLOGY:
1. Hct is the main determinant of blood viscosity, which is also affected by plasma
proteins, especially fibrinogen. As Hct increases, viscosity increases, elevating vascular
resistance and decreasing blood flow at any given perfusion pressure. As Hct rises above
60%, viscosity tends to increase exponentially. The effects of increased Hct on viscosity
are greater in the pulmonary circulation; in experimental animals, pulmonary vascular
resistance exceeds systemic vascular resistance when Hct >70%.
2. Clinically useful methods of measuring viscosity are currently not available.
Therefore, Hct is used as a proxy for viscosity. Because viscosity is affected by other
factors (e.g., flow rate in small vessels, plasma protein concentrations, acidosis, red blood
cell deformability), viscosity does not correlate directly with Hct.
3. Increased viscosity results in slowing of blood flow and sludging of red blood cells.
As blood flow is further reduced, occlusion of small vessels may result in ischemia and
consumption of platelets. Because glucose is transported mainly in plasma and relative
plasma volume is decreased, the decreased blood flow can result in hypoglycemia.
CLINICAL FINDINGS depend on organ system affected, as indicated below:
Organ system Signs & Symptoms
Skin Plethora, prolonged capillary filling time
CNS - Mild Lethargy, irritability, tremors, abnormal cry
CNS – Severe Apnea, seizures, hypotonia
Pulmonary Respiratory distress, cyanosis, persistent pulmonary hypertension
Renal Hematuria, oliguria/ anuria, renal vein thrombosis
Hematologic Thrombocytopenia, or other signs consistent with DIC
Gastro-intestinal Abdominal distension, blood in stools, necrotizing enterocolitis
Metabolic Persistent hypoglycemia
HEMATOCRIT SCREENING:
•Asymptomatic infants with no risk factors: Hct measurement is not necessary.
•Measure Hct in:
-Any infant at risk for polycythemia (see Introduction above)
-Any infant with symptoms that may be due to polycythemia
HEMATOCRIT SOURCE: Hct varies with source of blood. Capillary Hct (heel stick)
is 5-15% >venous. Arterial Hct averages 6% <venous. Decisions regarding treatment of
polycythemia should be made on the basis of venous Hct.
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Polycythemia/Hyperviscosity
5. Monitor vital signs throughout procedure and observe for catheter related problems. If
these occur, discontinue procedure and remove catheter.
6. After Partial ExTx, keep infant NPO for at least 4h. Give IV glucose water to
prevent hypoglycemia. If thrombocytopenia is present, keep infant NPO until
platelet count is in normal range. Repeat Hct measurement 4h after procedure.
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Neonatal Coagulation Disorders
intracranial hemorrhage. When blood loss is large, the infant may present with signs of
hypovolemia (pallor, weak pulses, tachycardia, hypotension, metabolic acidosis).
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Neonatal Coagulation Disorders
Factor Usual
Product Content Dose Indications
Fresh frozen All factors 10-20 mL/kg Disseminated intravascular coagulation
plasma (DIC); liver disease; protein C deficiency
Factor VIII Factor VIII 25-50 U/kg Factor VIII deficiency (Hemophilia A)
concentrate
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Neonatal Jaundice
PHYSIOLOGIC JAUNDICE (non-pathologic unconjugated hyperbilirubinemia):
1. Term Infants:
•50-60 % of all newborns are jaundiced in the first week of life.
•Total serum bilirubin peaks at age 3–5 d (later in Asian infants).
•Mean peak total serum bilirubin is 6 mg/dL (higher in Asian infants).
2. Preterm Infants:
•Incidence of visible jaundice is much higher than in term infants.
•Peak is later (5-7d).
•Because of ↑ risk of bilibubin encephalopathy (see below), “physiologic” jaundice is
more difficult to define and jaundice should be followed closely.
DEFINITION of NON-PHYSIOLOGIC JAUNDICE:
•Jaundice in the first 24 hours •Bilirubin rising faster than 5 mg/dL in 24 hours
•Clinical jaundice >1 week •Direct bilirubin >2 mg/dL
•In healthy term infants total serum bilirubin concentration >15 mg/dL
•Lower levels in preterm infants, “sick” infants, and hemolytic disease
(See section on Hemolytic Disease of the Newborn, P. 121)
BILIRUBIN METABOLISM: As red blood cells are lysed, they release hemoglobin.
Heme molecules (from hemoglobin) are converted to bilirubin. Bilirubin (unconjugated
or indirect) is bound to serum albumin and transferred to the liver where it is conjugated
to glucuronate by glucuronyl transferase. Conjugated (direct) bilirubin is excreted into
bile. A fraction of bilirubin from the stool is reabsorbed into the blood via the portal
circulation (enterohepatic circulation).
BILIRUBIN ENCEPHALOPATHY: The mildest form of bilirubin encephalopathy is
sensorineural hearing loss due to damage to the cochlear nuclei. Severe
encephalopathy causes kernicterus. Factors predisposing to neurotoxicity of
unconjugated hyperbilirubinemia include:
•When bilirubin concentration exceeds the binding capacity of serum albumin
•Displacement of bilirubin from albumin by acidosis or certain drugs (e.g.,
sulfonamides, ceftriaxone)
•Sepsis
•Preterm infants due to↑ risk due lower serum albumin concentrations and ↑ risk for
acidosis and sepsis.
CAUSES of UNCONJUGATED (INDIRECT) HYPERBILIRUBINEMIA:
1. Increased lysis of RBCs (i.e., increased hemoglobin release)
•Isoimmunization (blood group incompatibility: Rh, ABO and minor blood groups)
•RBC enzyme defects (e.g., G6PD deficiency, pyruvate kinase deficiency)
•RBC structural abnormalities (hereditary spherocytosis, elliptocytosis)
•Infection (sepsis, urinary tract infections)
•Sequestered blood (e.g., cephalohematoma, bruising, intracranial hemorrhage)
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•Polycythemia
•Shortened life span of fetal RBCs (80 vs. 120 d)
2. Decreased hepatic uptake and conjugation of bilirubin
•Immature glucuronyl transferase activity in all newborns: term infants have 1% of
adult activity, preterm infants have 0.1%.
•Gilbert Syndrome
•Crigler Najjar Syndrome (Non-hemolytic Unconjugated Hyperbilirubinemia):
inherited conjugation defect (very rare)
•Pyloric stenosis (mechanism is unknown)
•Hypothyroidism
•Infants of Diabetic Mothers (polycythemia is also common)
•Breastmilk Jaundice (pregnanediol inhibits glucuronyl transferase activity)
3. Increased enterohepatic reabsorption
•Breast feeding jaundice (due to dehydration from inadequate milk supply)
•Bowel obstruction
•No enteric feedings
EVALUATION of JAUNDICE (UNCONJUGATED)
1. Initial evaluation:
•Total and direct bilirubin •Blood type and Rh (infant & mother)
•Hematocrit •Direct Antiglobulin (Coombs) Test on infant
2. Later evaluation (as indicated):
•RBC smear, reticulocyte count (if evidence or suspicion of hemolytic disease)
•Blood culture, urinalysis, urine culture
•Thyroid function tests, G6PD assay, Hgb electrophoresis
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Hemolytic Disease of the Newborn
‡
With severe HDN, high quantities of antibody may block Rh antigen site resulting in an Rh+ infant
typing as Rh- and having a negative Direct Antiglobulin test.
(4) Intra-uterine Transfusion (IUT): When iso-immunization is severe, IUTs are
given to the fetus to prevent hydrops fetalis and fetal death. After multiple IUTs,
most of the baby’s blood will be Rh negative donor blood. Therefore, the Direct
Antiglobulin test will be negative, but the Indirect Antiglobulin Test will be positive.
After IUTs, the cord bilirubin is not an accurate indicator of rate of hemolysis or of
the likelihood of the need for post-natal exchange transfusion.
B. Minor Blood Group Incompatibility is uncommon, occurs in ~0.8% of pregnant
women and usually with E, c, Kell, Kidd or Duffy. Clinical presentation is similar to
Rh disease. Anti-Kell disease may be severe due to hemolysis or erythroid
suppression. Lewis antigen stimulates only IgM production, so maternal antibody
screen may be positive, but fetus is not affected.
C. ABO Incompatibility
(1) Genetics: With maternal blood types A and B, isoimmunization does not occur
because the naturally occurring antibodies (anti-A and -B) are IgM, not IgG. In type
O mothers, the antibodies are predominantly IgG, cross the placenta and can cause
hemolysis in the fetus. The association of a type A or B fetus with a type O mother
occurs in ~15% of pregnancies. However, HDN occurs in only 3%, is severe in only
1%, and <1:1,000 require exchange transfusion. The disease is more common and
more severe in African-American infants. Unlike Rh, ABO disease can occur in first
pregnancies, because anti-A and anti-B antibodies are found early in life from
exposure to A- or B-like antigens present in many foods and bacteria.
(2) Clinical presentation: generally less severe than with Rh disease.
(3) Laboratory findings that differ from Rh disease:
Smear: microspherocytosis
MCV <95, microcytic for a newborn (normal for adult)
Direct Coombs test is often weakly +.
MANAGEMENT:
A. Preparation prior to delivery should include:
-Blood: type O Rh negative packed RBCs, cross-matched against the mother. For
severe HDN, have blood in the Resuscitation Room to correct severe anemia
immediately after birth by partial exchange transfusion (ExTx). Anticipate
need for later ExTx for hyperbilirubinemia and have additional blood for these.
-Surfactant, if infant is preterm.
-Catheters (e.g., angiocaths) for immediate drainage of hydropic fluid.
B. Resuscitation: At birth, the major problems are cardiopulmonary and relate to
effects of severe anemia, hydrops and prematurity. Because of the multiple problems
with severe HDN, effective resuscitation requires several individuals.
-Obtain cord blood for bilirubin (total & direct), albumin, blood type & Rh, Direct
Coombs test, CBC, platelets, reticulocyte count and nucleated RBCs.
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Hemolytic Disease of the Newborn
-If the infant is hydropic, intubate immediately and begin assisted ventilation with
oxygen. If ventilation is difficult, drain pleural and ascitic fluid; during
paracentesis, take care to avoid puncturing the enlarged liver and spleen.
-Insert umbilical arterial (UAC) and venous catheters (UVC) (See section on
Umbilical Catheters, P. 25) and immediately measure blood pressures, arterial
pH and blood gas tensions, hematocrit (Hct) and blood sugar.
-Correct metabolic acidosis with alkali, but only if giving assisted ventilation (see
section on Acid Base Balance, P. 62).
-Correct anemia, which is essential for effective resuscitation.
•If arterial blood pressure is low, give simple transfusion of packed RBCs
(e.g., for Hct of 30%, push 10 mL/kg over 5 min; for Hct of 20%, push 10
mL/kg over 5 min, then repeat).
•Do not infuse packed RBCs or blood through UAC because of risk of
damage to spinal cord from emboli.
•With normal blood pressure, elevated central venous pressure, metabolic
acidosis or hydrops, correct anemia by partial ExTx (exchange 20 mL/kg,
then repeat hematocrit).
-Measure blood sugar frequently and correct hypoglycemia.
-Follow platelet counts; consider platelet transfusion for counts <50,000.
(C) Hyperbilirubinemia results from continued hemolysis and inability of the neonatal
liver to handle a large bilirubin load. Kernicterus (bilirubin encephalopathy) results
from high levels of indirect bilirubin (>20 mg/dL in a term infant with HDN).
Kernicterus occurs at lower levels of bilirubin in the presence of acidosis,
hypoalbuminemia, prematurity and certain drugs (e.g., sulfonamides).
-Measure bilirubin in cord blood and at least q4h for the first 12 to 24h. Plot
bilirubin concentrations over time. A graph for plotting bilirubin over time is
available at the unit clerk’s desk.
-Begin phototherapy shortly after birth. Although phototherapy may not eliminate
the need for ExTx, it may delay ExTx and decrease the number required.
-Use ExTx for hyperbilirubinemia not controlled by phototherapy (see P. 42).
•Indications depend upon absolute serum concentration of bilirubin, the rate of
rise of bilirubin, gestational age, albumin concentration and acid-base status. In
general, perform ExTx for cord bilirubin >5 mg/dL, for a rate of rise of bilirubin
>0.7 mg/h, and to prevent bilirubin >20 mg/dL in a term infant, and lower levels
in preterm infants (e.g., maintain serum bilirubin <10X the birthweight in kg).
•Blood should be reconstituted (to Hct ~40-50%) from fresh, O negative packed
RBCs cross-matched against the mother and type-specific fresh frozen plasma.
•Technique: About 30 min before ExTx, give albumin 1 g/kg to increase the
bilirubin bound to albumin in the circulation and make the ExTx more effective.
Exchange 2X the blood volume (estimate blood volume at 85 mL/kg).
Preferred technique is isovolumic ExTx, withdrawing blood from UAC and
infusing through UVC (with tip in IVC or low right atrium). Do not infuse
blood through UVC if tip is in portal circulation. Alternatively, ExTx can be
done through a single catheter (UAC or UVC) using aliquots <5% of the
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Hemolytic Disease of the Newborn
infant’s blood volume (e.g., 5 mL/kg). The blood should be warmed and the
bag agitated every few minutes (to prevent settling of the RBCs).
•Complications of ExTx:
-Hypocalcemia due to Ca++ binding by citrate. Give Ca-gluconate 100 mg
after every 100 mL of blood exchanged.
-Hypoglycemia, particularly after the ExTx, due to dextrose load from anti-
coagulant of donor blood and hyperinsulinism in HDN.
-Thrombocytopenia and granulocytopenia due to washout with the ExTx.
-Hyperkalemia, especially with older units of blood.
-Hypothermia, associated with inadequate warming of blood.
OUTCOME:
(A) Late anemia: Antibodies persist for weeks, cause continued hemolysis and can cause
anemia as late as age 6 months (especially in infants who had received IUTs). After
discharge, follow Hct weekly. Erythropoietin treatment will help prevent severe
anemia and further transfusions (see P. 111).
(B) Neurological prognosis is good. Commonest problem is sensorineural hearing loss.
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Bacterial Infections
Evaluation & Management: Early, rapid and thorough evaluation is essential for
successful treatment. An asymptomatic term or near-term newborn with even one risk
factor for sepsis requires careful physical examination and a screening complete blood
count (CBC) with differential and platelet count. In the presence of multiple risk factors,
also obtain a blood culture, and consider starting antibiotic therapy. For a preterm
infant with any risk factors, and for any symptomatic newborn, obtain CBC and blood
culture and start antibiotics.
Laboratory studies should include:
-CBC with differential and platelet count.
-Blood culture (aerobic culture system is sufficient for early onset sepsis).
-Lumbar puncture is essential for a symptomatic infant or asymptomatic infant
with a positive blood culture While it is optimal to obtain cerebrospinal fluid (CSF)
prior to starting antibiotics, do not delay antibiotic therapy if multiple attempts at
lumbar puncture are required. Evaluation of CSF should include culture, cell count
and differential, protein and glucose (with a blood glucose obtained at the same time).
-Serial serum C-reactive protein (CRP) levels are useful to rule-out early onset
sepsis in infants with GA ≤28 wks. If CRP is <1.0 mg/dL at 12 and 36 hours after
birth or onset of symptoms, the likelihood of proven or probable sepsis is only 0.3%.
However, with neutropenia, CRP is not reliable to rule-out sepsis.
-Chest radiograph for evidence of pneumonia.
-Tracheal aspirate for culture, if there are signs of respiratory disease.
Treatment: As soon as cultures have been obtained, begin antibiotic therapy. Ampicillin
and gentamicin IV are the currently recommended drugs for neonatal sepsis in this ICN.
For doses, see Table at end of this section.
Outcome: Early onset sepsis is associated with an increased likelihood of respiratory
distress syndrome, chronic lung disease, severe intraventricular hemorrhage, and
periventricular leukomalacia. Despite diagnostic and therapeutic advances, early onset
sepsis is associated with a high mortality and substantial morbidity; preterm newborns are
more severely affected. Among very preterm infants, mortality is about 35%.
LATE ONSET SEPSIS (After age 3 days):
Incidence among healthy term infants is much less than early onset sepsis. However,
preterm infants and term infants with various medical or surgical conditions are at greater
risk for late onset sepsis. More than 20% of infants with birthweight <1,500 grams will
have at least one episode of late onset sepsis.
Risk Factors for late onset bacterial infection are closely related to horizontal
transmission of causative organisms and include endotracheal intubation, indwelling
urinary and vascular catheters, especially venous catheters, lack of enteric feeding, and
exposure to broad-spectrum antibiotics, which may alter normal flora and permit
overgrowth and dissemination of fungal species and resistant bacteria.
Causative Organisms: In contrast to early onset infections, Gram-positive organisms
predominate and account for approximately 2/3 of cases. Coagulase-negative
Staphylococcus species (common skin flora) are the most common isolates, especially
among very preterm infants. However, Gram-negative bacteria (e.g., E. Coli, Klebsiella
pneumoniae, Pseudomonas aeruginosa) also cause a significant proportion of late onset
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Bacterial Infections
disease. Fungal infections (with Candida species) occur frequently in small preterm
infants (see section on Candidiasis, P. 128).
Presentation in most cases of late onset sepsis is gradual, rather than fulminant. The
first indications may be subtle signs such as feeding intolerance, need for increased
environmental oxygen, or persistent tachycardia. However, some infants become gravely
ill very quickly (especially with Pseudomonas infections), and the presentation may
include any signs mentioned above in Early Onset Sepsis.
Evaluation & Management: As with early onset sepsis, it is imperative to perform an
early and thorough diagnostic evaluation that should include blood culture, CBC with
differential and platelet count. Because CNS infection is more likely with late onset
sepsis, lumbar puncture with complete evaluation of CSF is essential (See above under
Early Onset Sepsis). Unlike early onset disease, urine infection is frequent. Urine
should be collected for urinalysis and culture. To prevent contamination of the specimen,
urine should be obtained by suprapubic needle aspiration. Urine by bag collection
should never be sent for culture. Also in contrast to early onset sepsis, serial CRP levels
may be useful to ruleout late onset sepsis in infants of any gestational age. If the CRP is
<1.0 mg/dL at 12 and 36 hours after the onset of symptoms, the likelihood of proven or
probable sepsis is 2.4%.
As soon as cultures have been obtained, antibiotic therapy should be instituted
without delay. While the spectrum of causative organisms differs from early onset
sepsis, ampicillin and gentamicin are appropriate initial antibiotic therapy.
Outcome: As with early onset infection, late onset disease is associated with significant
morbidity and mortality and preterm infants are more severely affected with a mortality
of up to 20%. Late onset sepsis is associated with an increased likelihood of patent
ductus arteriosus, bronchopulmonary dysplasia, necrotizing enterocolitis and death.
Age: 0 to 4 wk <1 wk ≥1 wk
BW: <1200 g 1200-2000 g >2000 g ≤2000 g >2000 g
Ampicillin* 25-50 q12h 25-50 q12h 25-50 q8h 25-50 q8h 25-50 q6h
Gentamicin* 2.5 q18-24h 2.5 q12h 2.5 q12h 2.5 q8-12h 2.5 q8h
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Candidiasis
-Bones and Joints: Warmth and swelling of the extremities in combination with
radiographic evidence of osteolysis or arthritis.
TREATMENT: Remove central venous catheter, unless blood stream infection clears
rapidly with antifungal therapy.
Amphotericin B, the gold standard for neonatal antifungal therapy, exerts its mechanism
of action and toxicity through binding to ergosterol in the cell membrane of fungal and
host cells, resulting in formation of membrane pores, cell depolarization followed by cell
death. Side effects include nephrotoxicity, hypokalemia, hypomagnesemia, anemia,
thrombocytopenia and infusion reactions (temperature and hemodynamic instability.
Liposomal Amphotericin B allows targeted antifungal therapy with less toxicity. The
drug is cleared through the reticuloendothelial system allowing higher liver and spleen
concentrations and reduced renal concentrations.
Flucytosine (5-FC) interferes with DNA synthesis. Because of toxicity and development
of resistant strains, it is of limited use in neonatal infections. However, if the infant can
tolerate oral medications, flucytosine is very useful for CNS infections and may act
synergistically with amphotericin B.
Fluconazole, a fungistatic drug, is the most effective of the azoles. Hepatotoxicity, the
main side effect, is transient and resolves with cessation of therapy. It has decreased
activity against C. glabrata and C. krusei.
Consultation with the Infectious Disease Service should be obtained for all neonatal
fungal infections except those limited to skin and mucous membranes.
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DIAGNOSTIC WORK-UP:
(A) Maternal: History of:
(1) Maternal serologies and when obtained
(2) Exposure to undercooked or raw meat, soil, animals
(3) History of sexually transmitted diseases (STDs) for mother and partners
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Congenital and Perinatal Infections
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Congenital and Perinatal Infections
HIV (cont’d):
-Consult with Immunology Service (476-9373)
-Begin AZT therapy according to current protocol.
>36 wks GA: 2mg/kg Q6h PO; <36 wk GA: Discuss with Immunology
-Age 1-2 wks: Immunology visit to adjust AZT dose as needed and repeat DNA PCR.
-Age 4-8 wks: Immunology visit to stop AZT after 2 negative DNA PCRs. Begin
PCP prophylaxis with Septra (75 mg/m2 BID 3 consecutive d/wk). Repeat DNA
PCR. If rapid weight gain occurs before 6 wks, recalculate dose.
-If persistently negative DNA PCR, repeat test at age 4 months of age.
-Confirm HIV status with ELISA at age 12 months.
-Note: Infant’s HIV status or exposure is confidential under California law.
Disclosure to biological father or others must be endorsed by the mother.
Breastfeeding is contraindicated with maternal HIV infection.
Circumcision is not contraindicated.
Hepatitis B Virus (HBV)
-Immunoprophylaxis:
-Infants of mothers who are known to be hepatitis B surface antigen (HbsAg)
negative should be immunized per the usual schedule of infant immunizations.
-Infants of mothers who are HBsAg positive: Give HepB Vaccine & hepatitis B
immune globulin (HBIG) before age 12 h. Complete the HepB Vaccine series in 1st
6 months*
-Infants of mothers with unknown HBsAg status:
A. Term infants:
-HepB Vaccine before age 12 h.
-If mother is found to be HBsAg positive, give HBIG before age 7 d**
-Complete HepB Vaccine series in 1st 6 months.
B. Preterm infants (BW < 2 kg):
-HepB Vaccine & HBIG before age 12 h
-If mother is found to be HBsAg positive: Complete 3 additional doses of
HepB Vaccine series per usual preterm schedule
*For preterm babies (BW< 2 kg), administer 3 additional vaccine doses per the usual
preterm immunization schedule.
**HBIG only effective if given within 7 d of birth. HepB Vaccine series is highly
effective alone.
Note: Breastfeeding by HBsAg positive mother is not known to increase risk of
transmission and, therefore, is not contraindicated
Hepatitis C Virus (HCV)
-No known immunoprophylaxis.
-Breastfeeding with HCV positive mother is not known to increase risk of transmission
and is not contraindicated. However, because HCV RNA and HCV antibody have
been detected in milk of mothers infected with HCV, transmission by breastfeeding is
theoretically possible. Therefore, the decision to breastfeed should be based on
informed discussion between a mother and her health care professional.
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Necrotizing Enterocolitis
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Necrotizing Enterocolitis
PREVENTIVE MEASURES:
-Intestinal priming (gut stimulation feedings): dilute, low volume feedings to
stimulate GI mucosal development
-Advance feedings slowly in small preterm infants (see section on Feeding of
Preterm Infants, P. 50).
-Do not advance feedings if there are gastric residuals, especially if bile stained
-Fresh human milk appears to be protective against NEC.
-Do not feed infants with PDA, UAC, or UVC.
-Do not give enteral feedings during and for 48-72h after indomethacin.
-Minimize antibiotic use, as they alter intestinal flora and select for resistant species.
-Epidemiologic controls: cohorting of multiple cases (possible infectious cause)
-Antenatal glucocorticoids for lung maturation also accelerate intestinal maturation.
-Suggested possible future approaches: enteral IgG/IgA, formula acidification,
anaerobic bacterial supplementation (bifidobacteria)
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Parenteral Nutrition
3. Fat: Intravenous lipid emulsions are essential components of TPN. They provide
essential fatty acids and are a concentrated energy source critical for growth and
development of infants not receiving enteral feedings. There are potential safety
concerns regarding administration of lipid emulsions to very low birth weight infants and
infants with hyperbilirubinemia, pulmonary hypertension and serious pulmonary disease.
To maximize benefits of lipids and minimize their adverse effects, use the following
guidelines: (a) provide sufficient lipids to prevent essential fatty acid deficiency; (b)
monitor for evidence of lipid intolerance; (c) adjust lipid dose based on clinical status.
•A lipid intake of 0.25-0.5 g/kg/d is required to prevent essential fatty acid deficiency.
•Include lipid emulsion in calculations of total fluid intake.
•Lipids yield 10 kcal/g.
•IV lipid preparations are available as a 20% soybean emulsion that yields 2 kcal/mL.
•See Table below for starting and advancing lipids.
•Deliver IV lipids over 24 hours.
•Do not allow lipids to exceed 60% of total caloric intake.
•Potential complications/risks include:
-Hyperlipidemia
-Potential risk of kernicterus at low levels of unconjugated bilirubin because of
displacement of bilirubin from albumin binding sites by free fatty acids. As a
general rule, do not advance lipids beyond 0.5 g/kg/d until bilirubin is below
threshold for phototherapy (see section on Jaundice, P. 118).
-Potential increased risk or exacerbation of chronic lung disease
-Potential exacerbation of Persistent Pulmonary Hypertension (PPHN)
-Lipid overload syndrome with coagulopathy and liver failure
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Parenteral Nutrition
Administration of Lipids
Initiate Advance by Goal is
Gestation Weight/Diagnosis (0.5 g/kg/d) 0.5 g/kg/d 3 g/kg/d by
Preterm <1,500 g, stable DOL 3 DOL 7 DOL 11
≥1,500 g, stable DOL 3 DOL 4 DOL 9
Very unstable DOL 3 When status improves
(e.g., severe RDS,
↑ bilirubin)
Term No pulm. disease DOL 3 DOL 4 DOL 9
Severe pulm. disease, Consider When status improves
PPHN, meconium at DOL 7
aspiration syndrome
(DOL, day of life; pulm., pulmonary; PPHN, persistent pulmonary hypertension of
newborn)
4. Electrolyte requirements are stated on the parenteral nutrition order form and must be
adjusted according to serum values and clinical condition. In utero accretion rates are:
•Calcium 3.3 mmol (130mg) /kg/d
•Phosphorus 2.39 mmol (74mg) /kg/d
•Magnesium 0.13 mmol (3.2mg) /kg/d
To maximize phosphorus and avoid precipitation, it may be necessary to give calcium by
peripheral IV bolus infusion. Consult the TPN order form for tables of solubility ratios
for calcium/phosphorus.
Goals for serum levels differ from those of older infants and children. Goals for
newborns are:
-ionized calcium: 1.2-1.4 mmol/L
-phosphorus: 6-8 mg/dL
-magnesium: 2-2.5 mg/dL
Start phosphorus at I mmol/kg/d. Do not start magnesium until its serum level has
decreased to <2.5 mg/dL.
Note: Initial PN solutions may be started without added electrolytes. Add electrolytes
gradually as the patient becomes more stable.
5. Acetate is metabolized to HCO3- and is added to adjust acid-base status. It can be
ordered as maximize, minimize, or balance with chloride depending upon infant’s pH.
6. Other Additives include:
•Trace elements are recommended as 0.2 mL/kg/d of trace element solution
containing zinc, manganese, copper, and chromium. See parenteral nutrition order
form.
-Preterm infants need additional zinc (300 mcg/kg/d) and selenium (2 mcg/kg/d).
-Term infants on TPN >4 weeks also need selenium (2 mcg/kg/d).
-In infants with cholestasis (i.e,. direct bilirubin >2.5 mg/dL), discontinue the
trace element solution and give:
Zinc 100 mcg/kg/d for term infants
“ 400 mcg/d TOTAL for preterm infants
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Neonatal Seizures
DEFINITION: A seizure is a paroxysmal behavior caused by hypersynchronous discharge of a
group of neurons. Neonatal seizures are the most common overt manifestation of neurological
dysfunction in the newborn.
DIAGNOSIS:
Seizure type Occurs in Clinical signs
Subtle Preterm and Term Eye deviation (Term)
Blinking, fixed stare (Preterm)
Repetitive mouth & tongue movements
Apnea
Pedaling, tonic posturing of limbs
Tonic Primarily Preterm May be focal or generalized
Tonic extension or flexion of limbs (often signals severe
ICH in preterm infants)
Clonic Primarily term May be focal or multifocal
Clonic limb movements (synchronous or asynchronous,
localized or often with no anatomic order to progression)
Consciousness may be preserved
Often signals focal cerebral injury.
Myoclonic Rare Focal, Multifocal, or Generalized
Lightning-like jerks of extremities (upper>lower)
Differentiation of Seizures from Nonconvulsive Movements:
-Jitteriness is distinguished clinically from clonic seizures by (1) no associated ocular
movements or autonomic phenomena, (2) stimulus sensitivity, (3) tremor that is suppressed by
flexing the limb.
- Distinguish benign neonatal sleep myoclonus (occurs in healthy newborns) from myoclonic
seizures.
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Neonatal Seizures
The duration of treatment following neonatal seizures is also determined by the underlying cause
(i.e., related to risk of recurrence), the physical examination and the EEG. The following
guidelines aim to continue Phenobarbital for the briefest time possible:
-When seizures have stopped and if the neurological examination is normal, consider stopping
Phenobarbital.
-If the neurological examination remains abnormal, then consider stopping medication if the
EEG is normal.
-Make this evaluation prior to discharge and then frequently after discharge, if the child has
been discharged on Phenobarbital.
-Stop Phenytoin when IV therapy is stopped as this drug is very difficult to maintain PO.
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Intraventricular Hemorrhage
MANAGEMENT: Other than early diagnosis and careful supportive care (including
correction of coagulopathies, circulatory and respiratory support), there is no therapy for
IVH. Consider consultation with Neurology for all IVH cases except Grade I and mild
Grade II. For progressive ventricular dilatation (post-hemorrhagic hydrocephalus),
the essential point is early recognition. Head circumference does not increase until after
there has been considerable ventricular dilatation. Therefore, do serial head U/S
examinations in infants with IVH ≥grade II. Some cases of ventricular dilatation will
respond to serial lumbar punctures and/or acetazolamide (carbonic anhydrase inhibitor)
or other diuretics (to decrease CSF production). Persistent, progressive ventricular
dilatation requires a ventricular reservoir or ventriculo-peritoneal shunt by a
neurosurgeon.
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Pain Management and Sedation
-Use appropriate equipment (smallest gauge needle, automatic heel lancet, etc.)
B. Treatment guidelines: Assess each infant on an individual basis. Using the NIPS, the
nurse and the medical team determine a pain score and the appropriate intervention for
pain management, as suggested by the guidelines shown in Table 2.
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Pain Management and Sedation
-Adjunctive Drugs (e.g., diazepam, midazolam, lorazepam, chloral hydrate) are useful
for sedation when pain is adequately managed.
SPECIFIC SITUATIONS for which analgesia or sedation is recommended and
suggested agent:
-Circumcision: EMLA (eutectic mixture of local anesthetics: lidocaine-prilocaine)
topically 1h prior to procedure, or dorsal penile block (1% lidocaine) immediately
before procedure.
-Lumbar puncture (analgesia when feasible): EMLA prior to procedure, or morphine
(0.05 – 0.1 mg/kg IV) at least 5 min prior to procedure.
-Nasal CPAP: Lidocaine jelly to nostrils q6h
-Non-emergent endotracheal intubation: Morphine (0.05 – 0.1 mg/kg IV) at least 5
min prior to intubation.
-Mechanical ventilation:Typical sedation involves one of the following:
•Morphine 0.05 - 0.1 mg/kg IV q 4-6h, or continuous infusion of 0.01-0.025
mg/kg/h. Titrate dose to lowest that achieves analgesic/sedative effect.
•Fentanyl 1-3 mcg/kg IV q1-2 h, or continuous infusion of 1-5 mcg/kg/hr. Titrate
dose to lowest that achieves analgesic/sedative effect.
•Phenobarbital is useful for long-term ventilation at 2.5 mg/kg IV or PO q12-24 h.
-Chest tube thoracostomy: Morphine 0.05-0.1 mg/kg IV. Attempt to give at least 5 min
prior to chest tube insertion. Unless it is a dire emergency, use local infiltration with
1% lidocaine.
-Venipuncture or minor procedures (IV catheter placement) may be painful,
especially if difficult to accomplish. Therefore, consider giving morphine 0.05 - 0.1
mg/kg IV at least 5 min prior to procedure.
-CT or MRI scanning: Sedation is often unnecessary, especially if infant has been fed
just before scan, is well-bundled, and efforts are made to minimize sound of scanning.
Otherwise, use pentobarbital (Nembutal™) 1.5-3.0 mg/kg IV immediately before
placing in scanner.
-Post-operative pain:
•Major surgery (e.g., thoracotomy, abdominal laparotomy): Morphine 0.1-0.2
mg/kg q3-4h IV prn for at least 24h after the operation. Discuss subsequent pain
medication with Neonatology Fellow and with Surgeons.
•Minor Surgery (e.g., hernia repair, pyloromyotomy): Acetominophen is usually
adequate. Discuss with Fellow and with Surgeons.
-Withdrawal of life support: The major aim is to relieve suffering as much as possible.
Discuss this with Neonatology Fellow and/or Attending. In many cases it is
appropriate to give relatively large doses of narcotics to alleviate suffering. In these
circumstances, morphine almost never causes apnea.
WEANING OF OPIATE ANALGESICS (morphine, fentanyl) should be done if the
agents have been given routinely for more than 3 d. Method of weaning depends upon
length of opiate therapy:
1. Short term therapy (<1 week): Initially reduce dose by 20%. Then reduce dose by
10% (of original dose) q6-8h. Discontinue drug as tolerated.
2. Long term therapy (>1 week): Reduce dose by 20% over first 24h. Then reduce dose
by 10% (of original dose) q12h as tolerated. Drug can usually be discontinued when it
is at about 20% of original dose, although subsequent small doses may be needed.
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Infants of Diabetic Mothers
Small left colon (Hypoplastic left colon syndrome) presents as lower bowel obstruction and
may be confused with Hirschsprung’s Disease. Cause is thought to be delayed innervation of
distal bowel. Diagnosis is made by barium enema and history of maternal diabetes. Condition
should clear within several days.
Polycythemia is associated with poor glycemic control or maternal vascular disease. (see section
on Polycythemia, P. 112)
Persistent pulmonary hypertension (see P. 91 for management)
Low cardiac output: IDMs who have had perinatal asphyxia with metabolic acidosis,
hypoglycemia and/or hypocalcemia may have cardiomagaly with ↓ contractility. This
responds to combined correction of all metabolic abnormalities.
Poor feeding is common. An IDM may take several days to establish nipple feedings.
MANAGEMENT of IDMs:
•Screen all IDMs for hypoglycemia, hypocalcemia and polycythemia, and treat appropriately.
•Careful examination and observation looking for conditions described above.
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Neonatal Hypoglycemia
BACKGROUND and PATHOPHYSIOLOGY: Glucose is the major energy source for
fetus and neonate. The newborn brain depends upon glucose almost exclusively. Up to
90% of total glucose used is consumed by the brain. Alternate fuels (e.g., ketones,
lactate) are produced in very low quantities. The usual rate of glucose utilization is 4-8
mg/kg/min. Glucose regulatory mechanisms are sluggish at birth. Thus, the infant is
susceptible to hypoglycemia when glucose demands are increased or when exogenous
or endogenous glucose supply is limited. Severe or prolonged hypoglycemia may
result in long term neurologic damage.
DEFINITION: Hypoglycemia in the first few days after birth is defined as blood glucose <40
mg/dL. In preterm infants, repeated blood glucose levels below 50 mg/dL may be associated
with neurodevelopmental delay.
ETIOLOGY: conditions associated with an increased risk for neonatal hypoglycemia include:
1. Decreased substrate availability:
•Intra-uterine growth retardation •Glycogen storage disease
•Inborn errors (e.g., fructose intolerance) • Prematurity
•Prolonged fasting without IV glucose
2. Hyperinsulinemia:
•Infant of diabetic mother •Islet cell hyperplasia
•Erythroblastosis fetalis •Exchange transfusion
•Beckwith-Wiedemann Syndrome •Maternal ß-mimetic tocolytic agents
•”High” umbilical arterial catheter •Abrupt cessation of IV glucose
3. Other endocrine abnormalities:
•Pan-hypopituitarism •Hypothyroidism
•Adrenal insufficiency
4. Increased glucose utilization:
•Cold stress •Increased work of breathing
•Sepsis •Perinatal asphyxia
5. Miscellaneous conditions:
•Polycythemia •Congenital heart disease
•CNS abnormalities
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Neonatal Hypoglycemia
MANAGEMENT OF HYPOGLYCEMIA:
•Glucometer reading >40 mg/dL and infant is feeding normally: follow usual nursery
protocol.
•Glucometer reading 20-40 mg/dL, infant is term and is able to feed:
-Draw blood for stat blood glucose.
-Feed 5 mL/kg of D5W.
-Repeat blood glucose or Glucometer 20 min after feeding.
•Glucometer reading: (a) <20 mg/dL or
(b) <40 mg/dL and NPO or preterm or
(c) <40 mg/dL after feeding or
(d) <40 mg/dL and symptomatic
-Draw blood for stat glucose measurement.
-Give IV bolus of 2-3 mL/kg of D10W.
-Begin continuous infusion of D10W at 4-6 mg/kg/min.
-If infant of diabetic mother, begin D10W at 8-10 mg/kg/min (100-125 cc/kg/d).
-Repeat blood glucose in 20 min and pursue treatment until blood sugar >40 mg/dL.
•For persistent hypoglycemia despite above measures:
-Increase rate of glucose infusion stepwise in 2 mg/kg/min* increments up to 12-15
mg/kg/min glucose. Use increased volume with caution in infants where volume
overload is a concern. Maximal concentration of glucose in peripheral IV is D12.5.
-If infant requires IV dextrose concentrations >12.5%, insert central venous catheter.
•Do not use D25W or D50W IV or large IV volume boluses as this creates rebound
hypoglycemia in infants who are hyperinsulinemic. In addition, administration of D25W or
D50W can cause dangerous increase in plasma osmolarity.
•If hypoglycemia is not controlled with above measures: Obtain Endocrine Consult to guide
further diagnostic evaluation and management. While awaiting consult, send blood (while blood
sugar is low) for glucose, plasma cortisol, growth hormone and insulin concentrations. Further
management may include glucocorticoids, diazoxide, somatostatin or pancreatectomy.
•Weaning IV dextrose infusion: When blood glucose has been stable for 12-24 h, begin
decreasing IV infusion by 1-2 mL/hr q3-4 hours if blood glucose remains ≥60 mg/dL.
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Inborn Errors of Metabolism
infancy, not with a specific laboratory abnormality, but with organomegaly, facial
coarseness and neurodegeneration and show a progressively degenerative course.
Peroxisomal disorders (e.g., Zellweger syndrome and neonatal adrenoleukodystrophy)
result from failure of the peroxisomal enzymes. They may present with features similar
to the lysosomal storage disorders. Common features of Zellweger syndrome include
large fontanel, organomegaly, Down-like facies, seizures and chondrodysplasia punctata.
Others include disordered steroidogenesis (congenital adrenal hyperplasia or Smith-
Lemli-Opitz), disorders of metal metabolism (Menkes syndrome, neonatal
hemochromatosis). Transient hyperammonemia of the newborn is more prevalent in
slightly premature infants receiving mechanical ventilation; onset is usually within the
first 24 hours of life. The ammonia level may be markedly elevated and dialysis may be
necessary. The cause is unknown and, if the newborn survives, there is no further
evidence of impaired ammonia metabolism.
CLINICAL FINDINGS suggestive of an IEM include:
-History of consanguinity, mental retardation, or SIDS; symptom onset with institution
of feedings or formula change; history of growth disturbances, lethargy, recurrent
emesis, poor feeding, rashes, seizures, hiccoughs, apnea, tachypnea.
-Physical findings: tachypnea, apnea, lethargy, hypertonicity, hypotonicity,
hepatosplenomegaly, ambiguous genitalia, jaundice, dysmorphic or coarse facial
features, rashes or patchy hypopigmentation, ocular findings (cataracts, lens dislocation
or pigmentary retinopathy), intracranial hemorrhage, unusual odors.
-Laboratory findings: metabolic acidosis with increased anion gap, primary respiratory
alkalosis, hyperammonemia, hypoglycemia, ketosis or ketonuria, low BUN,
hyperbilirubinemia, lactic acidosis, high lactate/pyruvate ratio, non-glucose-reducing
substances in urine, elevated liver function tests including PT and PTT, neutropenia and
thrombocytopenia.
INITIAL APPROACH:
-Rule out non-metabolic causes of symptoms such as infection or asphyxia.
-Obtain consult from Genetic/Metabolic Service
-Laboratory assessment prior to therapy:
• Blood: glucose, newborn screen, CBC with differential, platelets, pH and PaCO2,
electrolytes for anion gap, liver function tests, total and direct bilirubin, PT, PTT,
uric acid, ammonia. Other studies may be indicated as described in algorithm
below. Blood ammonia, lactate and pyruvate should be collected without a
tourniquet, kept on ice and analyzed immediately.
• Urine: color, odor, pH, glucose, ketones, reducing substances (positive for
galactosemia, fructose intolerance, tyrosinemia and others), ferric chloride reaction
(positive for MSUD, PKU and others) and DNPH reaction (screens for alpha-keto
acids). Amino and organic acids as described in algorithm below.
• CSF: glycine (for nonketotic hyperglycinemia), lactate, pyruvate if appropriate.
-Figures 1 and 2 are adapted from Burton BK: Inborn errors of metabolism in infancy: A
guide to diagnosis. Pediatrics 102:E69, 1998. These flow charts are guides to the
differential diagnosis of hyperammonemia (Figure 1) and metabolic acidosis (Figure
2 ) in newborns. Another helpful algorithm is in Rudolph’s Pediatrics, 20th ed., P. 292.
Not all inborn errors of metabolism will present with acidosis, hyperammonemia,
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Inborn Errors of Metabolism
NEONATAL HYPERAMMONEMIA
Absent Citrulline
Citrulline moderately Citrulline markedly
elevated, ASA present Elevated, no ASA
Urine Orotic Acid
CPS OTC
deficiency deficiency
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Inborn Errors of Metabolism
Abnormal Normal
organic acids organic acids
Abnormal
organic acids
ORGANIC Elevated pyruvate; Normal or low pyruvate;
ACIDEMIA normal L:P ratio elevated L:P ratio
Dicarboxylic
aciduria RESPIRATORY CHAIN
DEFECTS; PYRUVATE
CARBOXYLASE
Hypoglycemia DEFICIENCY
FATTY
ACID No hypoglycemia
OXIDATION METHYLMALONIC
DEFECTS ACIDEMIA;PROPIONIC
ACIDEMIA; MULTIPLE
CARBOXYLASE DEFICIENCY;
OTHERS
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Inborn Errors of Metabolism
OUTCOME: At present, for most IEMs, prognosis for survival or normal neurological
outcome is guarded, despite appropriate and aggressive therapy. It is likely that the
outcomes will improve with (a) presymptomatic diagnosis (i.e., by prenatal detection or
expanded neonatal screening), (b) identification of genes and other factors which impact
on phenotype, response to treatment, and outcome, and (c) alternative novel approaches
to therapy.
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Surgical Conditions
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Surgical Conditions
2. INTESTINAL OBSTRUCTION
A. Diagnosis and preoperative management:
•Intestinal obstruction should be suspected with maternal history of
polyhydramnios, large amount (>20 mL) of gastric fluid at birth, bilious or non-
bilious emesis, or progressive abdominal distension.
•Common causes include duodenal, jejunal, ileal, or colonic atresia, malrotation
with mid gut volvulus, meconium ileus with associated cystic fibrosis, meconium
plug, Hirschsprung’s disease, imperforate anus, and hypoplastic left colon.
•Infants with bowel atresia may pass meconium.
•The higher the obstruction, the more prominent is the vomiting. The lower the
obstruction, the more prominent is the distension.
•Make infant NPO, start IV, and monitor electrolytes, urine output and weight.
•Place Replogle tube to continuous suction and measure output.
•Obtain KUB looking for
-“double bubble” sign of duodenal atresia. If present, no further GI workup is
needed and patient should go to surgery when stable.
-multiple dilated loops of bowel indicating a more distal obstruction
-intraperitoneal calcifications suggestive of perforation with meconium ileus
-air throughout bowel to the rectum suspicious for Hirschsprung’s disease
-bubbly-appearing stool filling the bowel suggestive of meconium ileus and
cystic fibrosis
•Upper GI contrast study (with dilute Hypaque™or Gastrograffin™) may be
required to assess for malrotation and possible volvulus.
•Contrast enema using Gastrografin™ or dilute Hypaque™ may be done to identify
an area of obstruction or to relieve meconium plug or meconium ileus.
•Suspect acute volvulus secondary to malrotation if the baby has signs of shock,
metabolic acidosis or peritonitis. If there are signs suggesting volvulus,
emergency operation is indicated since gut viability may be threatened.
•Suspect Hirschsprung’s disease with repeated episodes of abdominal distension
or very delayed passage of meconium. Diagnosis can be made with suction rectal
biopsy. If no ganglion cells are seen, a surgical biopsy will confirm the diagnosis.
•Infants with Hirschsprung’s disease are at risk for development of fatal toxic
megacolon until the bowel has been decompressed by corrective surgery or
colostomy. Surgeons may choose to decompress initially with rectal irrigation.
This is different from simple enemas.
•Imperforate anus may be the sole abnormality or may be part of the VATER
association. Look carefully for evidence of recto-vaginal, recto-urethral or
perineal fistula. Ultrasound may help determine if the defect is low (and easily
repaired) or high (requiring colostomy drainage). These patients will need
eventual workup for tethered spinal cord and urinary tract anomalies.
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Surgical Conditions
B. Surgical management:
•If there is adequate space within the abdominal cavity, a primary closure of the
abdominal wall may be done, often with a gastrostomy tube for decompression.
Usually this is possible for omphaloceles, which tend to be small, and
occasionally for small gastroschises.
•Often a 2-stage procedure is required with the initial placement of a sterile
Goretex™ silo to cover the bowel. Each day, the silo is tightened by the
Surgeons so that the bowel is gradually pushed back into the abdominal cavity.
The final closure is performed in the operating room with removal of the silo and,
occasionally, placement of a mesh graft to help close the anterior abdominal wall.
•Risks of silo include infection and bowel necrosis. To decrease infection rate, the
silo should be closed within 4-7d.
•The surgeons will insert a central Broviac catheter for parenteral nutrition. This is
often not necessary with primary repair of small omphaloceles.
C. Post operative management:
•Check chest X-ray immediately post-operatively to evaluate lung fields and
position of catheter.
•If primary closure has been difficult or if a silo is used, the baby will require
assisted ventilation and will usually be kept on muscle relaxants for at least the
first few days after operation
•Ensure adequate ventilation and oxygenation by increasing inspiratory and end
expiratory pressures to maintain adequate lung volume and tidal volume as
abdominal girth increases secondary to capillary leak syndrome.
•Replacement of the bowel inside the abdomen and tightening of the silo will lead
to increased intra-abdominal pressure that will decrease diaphragmatic excursion
and make ventilation more difficult.
•The increased intra-abdominal pressure may also lead to decreased urine output.
•Provide adequate pain relief, usually by continuous infusion of morphine.
•Maintain good hydration and avoid hypotension postoperatively to ensure adequate
bowel perfusion. For the first 24h postoperatively, use D10% Lactated Ringer’s
solution at 1.5 times maintenance (i.e., 150 mL/kg/d). Frequent extra boluses of
normal saline may be required to maintain adequate urine output. Consider early
use of dopamine.
•Continue preoperative antibiotic therapy.
•After 48 hours postoperatively, consider starting central parenteral nutrition (see P.
136 for guidelines).
•Maintain Replogle tube to continuous suction, record volume of fluid drainage,
and replace with 0.9% NaCl if the amount exceeds 10 mL/kg per 12h shift.
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•If infant is receiving muscle relaxants, insert indwelling urinary catheter for
accurate measurement of urine output.
•When infant is ready to start enteral feedings, use an elemental formula such as
Pregestimil™, start with small volumes and advance slowly (see section on
Feeding of Preterm Infants, P. 50). Infants with gastroschisis are at very high risk
for necrotizing enterocolitis (see P. 133).
•Because infants with gastroschisis are also at increased risk for intestinal atresia,
observe infant closely for abdominal distension as feedings are advanced.
•Infants who require secondary closure of their abdominal wall defect will usually
not be on full enteral feedings until at least 3 weeks postoperatively.
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Meningomyelocele
INTRODUCTION: Failure of closure of the neural tube during the third week of
gestation leads to the constellation of defects observed in patients with
meningomyelocele (MMC). The open neural tube is continuous with the surface of the
skin. For this reason, infants with MMC are at risk for bacterial meningitis due to the
spinal defect. Leak of cerebrospinal fluid (CSF) leak is commonly observed. The major
indication for early operative repair (within 48h of delivery) is prevention of infection.
Although protection of the exposed neural tissue from trauma and drying is essential, the
neurological deficit caused by MMC is fixed and rarely improves following repair.
Deterioration, however, can occur.
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Meningomyelocele
POST-OPERATIVE MANAGEMENT:
•After surgical repair, a dry Telfa™ dressing should be applied to the incision daily or
PRN if soiled. Gently clean the incision with sterile normal saline and apply a layer
of bacitracin ointment. Place Duoderm™ around the incision and use paper tape to
prevent skin breakdown. DO NOT use Tegaderm™ or Opsite™ post-operatively.
Observe carefully for signs of wound infection or CSF leak.
•Discuss orders with Neurosurgery regarding positioning of infant, antibiotics, feeding,
and timing of post-operative cranial ultrasounds.
•Obtain abdominal and hip ultrasound and request Urology and Orthopedic consults for
evaluation of urinary function and associated orthopedic abnormalities.
Prior to discharge, arrange with Neurosurgical Nurse Specialist for patient to be enrolled
in Spina Bifida Clinic.
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Fetal Therapy
DEFINITION: A therapeutic intervention for the purpose of correcting or treating a fetal
anomaly or condition. In almost every case, the fetus is at risk of intrauterine death from
the abnormality.
Urinary Tract Obstruction: Complete obstruction of the fetal urinary tract results
in severe renal damage as well as pulmonary hypoplasia from severe
oligohydramnios. Despite early enthusiasm for fetal decompression of the urinary
tract, fetal intervention has seldom been beneficial and is now rarely performed.
Fetal Tumors Causing Hydrops Fetalis: When the relatively rare fetal tumors,
congenital cystic adenomatoid malformation of the lung (CCAM) and
sacrococcygeal tumor (SCT), are associated with hydrops fetalis, fetal mortality
approaches 100%. These tumors cause hydrops by either venous obstruction due to
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Fetal Therapy
mediastinal shift (CCAM) or high output heart failure (SCT). Operative removal of
these tumors has resulted in survival of ~50% of the affected fetuses.
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Hydrops Fetalis
INTRODUCTION: Hydrops fetalis is an excess accumulation of fluid in the fetus.
Depending on the severity and cause of hydrops, there may be edema of fetus and
placenta, ascites, pleural effusions and/or pericardial effusions. In previous years, most
cases of hydrops were caused by severe erythroblastosis fetalis secondary to Rh iso-
immunization (see Hemolytic Disease of the Newborn, P. 121). With the marked
decrease in this condition (due to prophylaxis with immune globulin), most cases of
hydrops fetalis are now caused by other conditions and are known as non-immune
hydrops. The rest of this section deals only with non-immune hydrops fetalis.
MANAGEMENT:
1. Antenatal management should be directed towards making a diagnosis, with the aim
of identifying those in whom either prenatal or immediate post-natal intervention may be
effective. Diagnostic techniques include fetal ultrasonography, fetal echocardiography,
examination of maternal blood for fetal erythrocytes (Kleihauer-Betke test),
amniocentesis and sampling of fetal blood. In some cases, fetal intervention is effective
(e.g., fetal transfusion for anemia due to Parvovirus B19 infection, treatment of fetal
tachycardia). In others, delivery corrects the underlying problem (e.g., chorioangioma of
placenta). Very few hydropic infants survive if delivered before 30 weeks of gestation.
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Hemolytic anemia
-α-thalssemia Lymphatic malformations
-RBC enzyme deficiencies -Pulmonary lymphangiectasis
Other anemias -Cystic hygroma
-Feto-maternal hemorrhage -Multiple pterygium syndrome
-Twin-twin transfusion (donor) -Noonan syndrome
Chest masses
Cardiac
-Cystic adenomatoid malformation
-Fetal arrhythmias
-Diaphragmatic hernia
-Premature closure of foramen ovale
-Pulmonary sequestration
-Hypoplastic left heart
-Intrathoracic mass
-Hypoplastic right heart
-Ebstein’s anomaly of tricuspid valve Skeletal conditions
-Cardiomyopathy -Asphyxiating thoracic dystrophy
-Cardiac tumors -Osteogenesis imperfecta
-Premature closure of ductus -Chondrodysplasia
arteriosus Genetic metabolic disease
-Other structural anomalies -Gaucher Disease
Chromosomal abnormalities -Mucopolysaccharidosis
-Trisomy 21, 18 -Nieman-Pick Disease
-Turner syndrome -Neonatal hemochromatosis
Infections Fetal Hypomobility
-Viral (Parvovirus B19, Herpes, -Arthrogryposis
CMV) -Neu-Laxova syndrome
-Toxoplasmosis -Pena-Shokier syndrome
-Syphilis -Myotonic dystrophy
-Chagas Disease CNS anomalies
Vascular malformations -Absent corpus callosum
-Chorioangioma (placenta, umbilical -Encephalocele
vessels) -Holoprosencephaly
-Liver hemangioma Other
-Cerebral A-V malformation -Bowel obstruction with perforation
-Sacrococcygeal teratoma (meconium peritonitis, volvulus)
-Klippel-Trenaunay syndrome -Infant of diabetic mother
Vascular accidents -Prune belly syndrome
-Intracranial hemorrhage -Congenital nephrosis
-Thrombosis of renal veins, IVC -Maternal indomethacin therapy
-Twin-twin transfusion (recipient)
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Intensive Care Nursery House Staff Manual
Multiple Births
INTRODUCTION: Multiple gestations are high-risk pregnancies. The rate of
monozygotic (MZ) twins is relatively constant at 3-5/1000 deliveries, whereas the
dizygotic (DZ) twinning rate varies from 4-50/1000 deliveries and is influenced by race,
heredity, maternal age, parity and nutrition. The incidence of multiple births is increasing,
partly due to older maternal age and use of assisted reproductive technology.
ZYGOSITY and PLACENTATION: MZ twins result when a single ovum is fertilized
and subsequently divides into two embryos. The placenta-membrane relationship,
determined by timing of the division (Table 1), may be dichorionic-diamnionic (di-di),
monochorionic-diamnionic (mono-di), or monochorionic-monoamnionic (mono-mono).
Conjoined twins are very rare and occur when the embryo incompletely divides after the
13th day of fertilization. DZ twins develop from two fertilized ova and the placenta is
always di-di. Higher-order fetuses may be either MZ or multizygotic. The perinatal
mortality rate is closely related to type of placentation (Table) with mono-mono twins at
highest risk. Zygosity can be determined for most twins by placentation, gender and
blood type. Immunologic studies or DNA analyses can prove zygosity.
Table. Zygosity and placentation.
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Multiple Births
leads to anemia in the donor and polycythemia in recipient. Acute and massive shift of
blood may occur via A-A or V-V anastomoses when perfusion pressures become
unbalanced at birth or after demise of one fetus. Demise of one twin places the survivor
at risk for exsanguination, acute hypotension and embolization (which may lead to CNS
lesions), limb amputation, intestinal atresia and gastroschisis. TTTS complicates 5-15%
of all monochorionic twins and has 80-100% mortality without treatment and a 15-50%
risk of handicap in survivors. Significant growth discordance (>25% disparity in
weights) increases perinatal mortality. In severe cases, the SGA fetus is compacted into a
small, oligohydramniotic sac, and is referred to as a “stuck twin,” in whom pulmonary
hypoplasia is common. Cord entanglement and knotting are frequent in mono-mono
twins, which accounts for their high mortality rate (50-60%). Diagnosis of TTTS is
suggested when monochorionic twins show a hemoglobin difference of >5 gm/dL.
Vascular communications can be demonstrated during pregnancy by ultrasonography or
by examination of placenta at birth. Currently, UCSF is participating in a multi-center
trial of obstetrical intervention for TTTS (see section on Fetal Therapy, P. 166).
PEDIATRIC MANAGEMENT of MULTIPLE BIRTHS:
-Obtain information needed for preparing for resuscitation including number of fetuses,
gestational age, estimated fetal weights, type of placentation, presence of anomalies and
complications (e.g., TTTS, hydrops, stuck twin).
-A pediatric team with personnel assigned to each infant should attend the delivery
and be prepared to resuscitate infants with asphyxia (the 2nd born twin is at greater risk
for asphyxia) and other identified complications and anomalies.
-Examine infants for signs of prematurity, growth retardation and anomalies. Rapidly
measure chem strip and hematocrit and begin appropriate therapy.
-If TTTS is suspected, have whole blood or PRBCs cross-matched against the mother
available in resuscitation room. Measure arterial and central venous pressures and
arterial pH and blood gas tensions immediately after initial resuscitation to assess the
circulatory status. If polycythemia is present, reduce hematocrit (see Polycythemia, P.
112). Management of the anemic donor is less straightforward. Recent acute blood loss
requires the same management as other hypovolemic infants (see Neonatal Shock, P.
101). The donor with prolonged, severe anemia may not tolerate blood volume
expansion. In this case, perform partial exchange transfusion with PRBCs to raise
hematocrit (see section on Neonatal Anemia, P. 108).
OUTCOME:
Perinatal mortality and morbidity for twins are five times higher than for singletons.
Prematurity, low birth weight and TTTS are the major contributors. Higher-order
multiples deliver earlier and, therefore, are at even greater risks. Twins, especially MZ,
have higher incidences of cerebral palsy and mental retardation.
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Perinatal Substance Abuse
Respiratory
-Apnea -Tachypnea
-Yawning
Gastrointestinal
-Ineffective feeding -Excessive sucking
-Diarrhea -Hyperphagia
Note: During withdrawal from maternal methadone, symptoms may be later in onset
(several days) and may last weeks.
2. Medical Management of opiate withdrawal: The goal is to maintain infant comfort
and enable the infant to feed, sleep, and gain weight appropriately. Withdrawal
scoring systems are used to assess severity of withdrawal and to help guide
treatment. Management combines behavioral and soothing methods with
pharmacologic interventions when necessary.
• Behavioral & soothing: swaddling, rocking and reduced environmental stimulation
• The mainstay of pharmacologic treatment for opiate withdrawal is treatment with
opiates, either alone or in combination with other medications. Medications used
are dilute tincture of opium (DTO), phenobarbital, and benzodiazepines.
Dosage is titrated according to severity of withdrawal using a scoring system.
-DTO: Usual starting dose is 0.1 mL/kg PO q3-4h. Increase dose by 0.05 to 0.1
mL increments until symptoms are controlled. Usual dose for withdrawal at birth
ranges from 0.2 to 0.5 mL q3-4h. Higher doses may be necessary to control
significant physiologic signs including diarrhea, pyrexia, hypertension and
hypertonicity.
-Phenobarbital does not adequately treat diarrhea and seizures and should not be
used as the sole treatment for withdrawal.
-Diazepam can be a useful adjuvant drug but should not be used as the sole
medication. Usual dose is 0.1 mg/kg PO q6h prn to decrease irritability and
increase infant comfort.
3. Weaning of treatment medication: Once DTO has been titrated to a level that
controls the symptoms of drug withdrawal, a judicious weaning of medication should
begin. A common method is to decrease the dose of DTO by 10% (every day or every
2 days), with continued surveillance of the infant for tolerance of this decrease. The
goal of weaning the medication is to allow the infant to acclimate to the lower dose
while assuring that the infant is consolable and is able to sleep, eat, and gain weight
appropriately. Objective measurements using a drug withdrawal scoring system
should be used to evaluate the rate and success of weaning of the medication.
B. Cocaine increases maternal arterial blood pressure, decreases uterine blood flow, and
transiently increases fetal systemic blood pressure. Perinatal complications associated
with cocaine use include:
-Spontaneous abortion and stillbirths -Placental abruption
-Preterm labor and delivery -Intrauterine growth retardation
-Fetal hypoxemia and distress -Fetal vascular accidents
Cocaine-exposed infants manifest neurobehavioral abnormalities initially described as drug
withdrawal, but are more likely due to acute intoxication, including:
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C. Alcohol: Incidence of fetal alcohol syndrome (FAS) in the United States ranges from
2-5/1,000 live births and is highest among women who report “heavy” drinking. Accurate
incidence and prevalence rates of FAS are difficult to obtain because the diagnosis is
often missed in the neonatal period; most cases are diagnosed after the age of 6 years.
Diagnosis of FAS is by history and physical examination. There are no laboratory tests
to identify or quantify alcohol exposure. FAS has three main features:
-Growth retardation (prenatal and postnatal)
-Facial features:
•Short palpebral fissures •Midface hypoplasia
•Flat, broad nasal bridge •Broad philtrum
•Thin vermillion border • Low set, dysplastic ears
•Ptosis •Strabismus
-CNS abnormailities:
•Microcephaly • Neurosensory hearing loss
•Dysgenesis of corpus callosum •Hypoplasia of basal ganglia and cerebellum
•Hypotonia •Feeding difficulties
Long term problems include: attention-deficit hyperactivity disorder, speech and
behavioral problems and learning disabilities.
D. Amphetamines, like cocaine, cause sympathomimetic effects in the mother and fetus.
Signs in the newborn are similar to those for cocaine exposed infants. In some cases,
there may be increased metabolic rate with very large insensible water loss. Perinatal
complications associated with use of amphetamines include:
-Preterm labor and delivery -Intrauterine growth retardation
-Intracranial hemorrhage -Strokes
E. Cigarettes and Nicotine are the drugs most often used during pregnancy. Nineteen
percent of pregnant women between ages 15 to 44 years smoke. Perinatal complications
occur in a dose-dependent fashion. Cigarette smoking represents the most influential,
identifiable and common factor adversely affecting perinatal outcomes. Maternal
smoking increases risk for:
-Spontaneous abortion and stillbirth -Placental abruption
-Fetal growth retardation -Prematurity
-Sudden Infant Death Syndrome (SIDS) -Asthma and otitis media during infancy
Nicotine concentrates in fetal blood, amniotic fluid and breast milk. Nicotine in fetal
blood and amniotic fluid may exceed maternal concentrations. The mechanism of adverse
effect on pregnancy is unknown but possibilities include decreased uterine blood flow,
increased fetal carbon monoxide, fetal hypoxemia, disturbed protein metabolism and
effects from other toxic substances in cigarette smoke.
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Renal Disorders
ACUTE RENAL FAILURE (ARF) , defined as a serum creatinine >1.5 mg/dL, occurs
in 6-23% of ICN patients and is described as either oliguric ARF (urine output <1
mL/kg/hr) or non-oliguric ARF (urine output is maintained despite decreased glomerular
and tubular function). There are three categories of ARF:
•Functional (Prerenal): due to ↓ renal perfusion, not the kidney itself
•Intrinsic (Renal): usually renal tubular dysfunction.caused by an acute insult
•Obstructive (Postrenal): due to anatomic urinary tract obstruction
1. Findings in ARF:
A. Clinical signs associated with ARF include:
•Oliguria or anuria •Hematuria, proteinuria
•Fluid overload •Hypertension
•Cardiac dysrhythmias (with ↑ K+)
B. Laboratory findings in ARF include:
•Creatinine > 1.5 mg/dL (An elevated creatinine on the first post-natal day is more
likely due to elevated maternal creatinine or increased production of creatinine
from tissue breakdown.)
•Abnormal electrolytes (especially, ↑ K+)
•↑ BUN
C. Initial Evaluation of ARF:
•Careful perinatal & neonatal history
•Physical examination for signs suggestive of renal disease (see above)
•Abdominal ultrasound
•Laboratory tests:
-Serum: electrolytes, BUN, creatinine, CBC, pH and blood gas tensions
-Urine: pH, urinalysis, culture, gram stain Na+, K+, Cl-, osmolality
D. Diagnostic Indices in ARF (useful in determining type of ARF):
Findings in
Test Pre-Renal ARF Renal ARF
Urine osmolality (mmol/L) >400 <400
Urine Na+ (mEq/L) 31 ± 19 63 ± 35
Urine/Plasma Creatinine 29 ± 16 10 ± 4
Fractional excretion of sodium <2.5 >2.5
(FENa, %)
[Urine Na+] [Serum Creatinine] x 100
[Serum Na+] [Urine Creatinine]
2. Causes of ARF:
A. Functional Renal Failure, the commonest type, is characterized by inadequate
renal perfusion, and is often called prerenal azotemia. Causes are any conditions
that lead to inadequate renal perfusion (e.g., dehydration, hypovolemia, shock,
myocardial failure, patent ductus arteriosus). Treatment is correction of the
underlying cause and supportive care.
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Renal Disorders
B. Intrinsic Renal Failure: The most common cause is acute tubular necrosis (ATN)
resulting in renal tubular dysfunction. ATN may be precipitated by shock,
prolonged prerenal state, or nephrotoxic drugs. Oliguria or anuria is prominent.
With recovery, there may by polyuria with dehydration and electrolyte disorders.
Treatment includes:
•Strict I & O (urinary catheter)
•At onset, consider fluid challenge (10-20 mL/kg) to R/O functional ARF
•If no response to fluid challenge, restrict intake to insensible water loss and
urine output.
•Consider one dose of furosemide
•Consider low dose dopamine (2-4 mcg/kg/min) to ↑ renal blood flow
•Restrict intake of K+ and PO4
•D/C any nephrotoxic drugs
•Obtain Nephrology Consult
•Dialysis as needed
C. Obstructive Renal Failure is due to bilateral urinary tract obstruction (in males
usually posterior urethral valves). Obstruction in fetal life can result in cystic
dysplasia of the kidney. Treatment consists of diagnosis and relief of the
obstruction and careful supportive care. With relief of obstruction, there may be
polyuria with electrolyte disorders. Infection is common and patients receive
urinary antibiotic prophylaxis.
OTHER NEONATAL RENAL DISORDERS:
1. Renal tubular acidosis (RTA), caused by defects in reabsorption of HCO3- and
secretion of H+ ions, generally presents metabolic acidosis and inappropriately high urine
pH (>6.0). This occurs frequently in preterm infants and is transient. RTA can also be
associated with a wide variety of other conditions.
2. Syndrome of Inappropriate Secretion of Antidiuretic Hormone (SIADH) is a very
infrequent disorder of fluid and electrolyte balance due to excessive release of
antidiuretic hormone leading to water retention and hyponatremia. Findings include
oliguria, low serum osmolality, hyponatremia, concentrated urine and elevated urine
sodium. Treatment is strict restriction of fluid intake.
3. Renal Cystic Disease: There are two types:
A. Autosomal recessive polycystic kidney disease presents with abdominal masses,
hypertension, renal insufficiency, hepatic and biliary fibrosis and there may be
pulmonary failure from lung hypoplasia.
B. Autosomal dominant polycytic kidney disease usually has its onset in the 3rd to
5th decade of life. In some newborns, it can present with bilateral flank masses,
hypertension, renal insufficiency and cystic involvement of other organs.
4. Congenital nephrotic syndrome may be secondary to congenital infections, but most
commonly is Finnish-type congenital nephrotic syndrome, an autosomal recessive
disease. These infants present with proteinuria that may be severe enough to cause fetal
hydrops.
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Intensive Care Nursery House Staff Manual
Blood Tests:
•pH and blood gas tension measurements
-for patients in ICN*
-at intensive resuscitations (“set-ups”) in the Neonatal Resuscitation Room in
the Obstetrical Delivery Suite
-on umbilical arterial and venous blood from doubly-clamped segment of
umbilical cord for all infants born at UCSF
•Electrolytes (Na+, K+, Cl-, and ionized Ca++)*
•Hematocrit (Hct, spun)
•Hemoximetry (oxyhemoglobin saturation, total Hgb, Met-Hgb and CO-Hgb)
•Activated clotting time (ACT, for ECMO patients only)
*Results for these tests are reported to the infant’s bedside Nurse within 15 min after the
sample is obtained.
Other Tests:
•Neonatal pulmonary function testing (pulmonary mechanics only). Ask the
Neonatology Fellow to request this test.
•Neonatal hearing screening: This is done prior to discharge on all ICN and Well
Baby Nursery infants. Results are reported in the infant’s chart.
Other services: The staff of the NCPL also is responsible for:
•Pulse oximetry monitoring
•Transcutaneous CO2 monitoring
•Maintaining the ICN Database and the Obstetrical Database
For any questions about the NCPL, contact Mr. Gene Qin, Supervisor of the NCPL, or
Ms. Mureen Schlueter, Manager of the NCPL.
NCPL phone: 353-1755
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