Initial Care of Newborn  Other persons needed to prepare medication doses and record

Dr. E. Posecion interventions and times

June 18, 2013
Group 5 Equipment
 Equipment and supplies must be available at every delivery
Perinatologist – keeps the baby healthy inside  All delivery settings should have equipment for O2 administration,
Neonatologist – concerns with high risk pregnancy bag and mask ventilation and ET intubation
 CS delivery rooms should be stocked with supplies for vascular
Successful Approach to Neonatal Resuscitation access, volume expanders and medications as well as respiratory
 Anticipation equipment
- Personnel  Low risk labor-delivery-recovery rooms may be served by ER charts
- Equipment  Correct size respiratory equipment should be unpacked, prepared
 Recognition and tested
 Techniques in resuscitation  Responsibility for restocking of supplies and routine maintenance
of equipment must be clearly designated
Risk Factors Associated with the Need for Resuscitation
Maternal Recognition
 PROM – high risk for infection  Three key steps
 Bleeding in 2nd or 3rd trimester – hypotensive mother, no blood o Evaluate infant
supply for the baby o Decide on the correct action
 Severe PIH – blood vessels are narrowed o Take that action
- Premature baby  Three basic signs forms basis for evaluation
 Chronic HPN o Respirations
 Previous fetal or neonatal death o Heart rate
 Pharmacologic Therapy o Color
 Chronic illness  Techniques in resuscitation
 Maternal infection A - Establish an Airway
 Heavy Sedation Positioning
 No prenatal care Suctioning
 Substance abuse B - Initiate Breathing
 Diabetes Mellitus Tactile stimulation
Positive-pressure ventilation
Fetal C - Maintain Circulation
 Multiple gestation – 2nd baby is preterm Chest compressions
 Preterm gestation (<35 wks) and Post-term gestation (>42wks) Medications and volume expanders
 Size-date discrepancy
 Congenital abnormalities
Physical Examination of the Newborn
 Rh isoimmunisation/hydrops fetalis
 Polyhydramnios and oligohydramnios
 Thorough physical examination is essential within the first 24 hours
 Reduced fetal movement before onset of labor
of life to identify problems and institute early intervention
 Intrauterine infection
 Any major abnormalities should be discussed with the parents as
 IUGR
soon as feasible
 Normal newborn should also be examined by the physician within
Intrapartum
24 hours before discharge
 Fetal distress
 High risk infants should be assessed immediately by the nursing
 Prolapsed cord
staff and the physician
 Prolonged labor
 Precipitous labor Vital Signs
 Forceps delivery  Nursing personnel should measure the following within the 1st hour
 CS delivery of life
 Narcotic administration to mother within 4 hours of delivery o Temperature
 Abnormal presentation o Respiratory rate
 Prolonged rupture of membranes o Heart rate
 Antepartum haemorrhage
 Thick meconium staining Gestational Age Assessment
 Nonreassuring FHR patterns  Physicians perform gestational age assessment as part of their
 Vacuum-assisted delivery evaluation
 Compare with maternal estimated date of confinement (by UTZ
Successful Approach to Neonatal Resuscitation and LMP)
Personnel
 There should be one person solely responsible for the baby who is Body Measurements
capable of initiating resuscitation  Nursing staff should record the infant’s
 A second person with complementary skills should be available o Weight (in Kg)
 o Length (in cm) - Congenital hypothyroidism
o Head circumference (in cm) - Congenital adrenal hyperplasia
 These should be plotted on the growth chart - Galactosemia
 This facilitates identification of infants who are small or large for - G6PD deficiency
gestational age, or microcephalic or macrocephalic
Management of the High Risk Infant During Transition
Routine Evaluation During Transition
 Respiratory Areas
o Is there any evidence of increasing respiratory distress?  Monitoring
o Is there tachypnea without grunting? o Cardiorespiratory monitor
o Is the infant pink and well saturated? o Blood pressure readings
o Mild grunting during the first few minutes of life is normal o Arterial pressure if with arterial catheter
o Increasing grunting at 15 to 30 min of age and associated with o Pulse oximeter
other signs of respiratory distress is abnormal (pneumonia,  Vascular access
RDS, pneumothorax)  Oxygen and ventilator support
o Tachypnea with grunting – TTN  Evaluation of suspected sepsis
o All infants with any signs of respiratory distress should be
placed on a pulse oximeter to assess O2 saturation
 Cardiovascular NEWBORN SCREENING
o Is the infant well perfused? What is the Newborn Screening Test?
o Does the infant have a murmur?  Has been an important part of routing newborn care in
o Hypoperfusion often accompanies either sepsis or significant developed countries
asphyxia  Introduced in the Philippines in 1996
o Murmurs maybe present in healthy newborns but in the  A series of tests used to detect a group of silent disease
presence of cyanosis, poor perfusion or pulses, consider the present at birth which if undetected late could lead to
presence of cardiac disease development delay or death
 Neurologic Newborn Screening
o Is the infant lethargic and hypotonic or is the infant jittery?  A public health program for the early identification of disorders
o Lethargy and hypotonia are associated with both sepsis and that can lead to mental retardation or death
asphyxia  An integral part of routine newborn care in most developed
o Jitteriness may indicate early drug withdrawal or countries
hypoglycaemia  As routine as Vit K injection or cord care
o Coarse high-amplitude jitteriness is sometimes seen in infants  In the Philippines, it is recognized as part of the standard newborn
with hypoxic-ischemic encephalopathy care
 Temperature
o Temperature must be followed closely in preterm infants Why is it important?
o Because of a larger surface to volume ratio, they are more  Babies with these metabolic disorders look normal at birth
likely to quickly become hypothermic  One will never know that the baby has the disorder until the onset
 Laboratory evaluation of signs and symptoms which may already be irreversible such as
o Blood glucose mental retardation and death.
- Prematurity
- Respiratory distress  Newborn screening is ideally done on the 48th hour or at least 24
- Suspected sepsis hours from birth.
- Hypovolemia/hypotension  Sick and premature babies must be screened by the 7th day of life
- Maternal diabetes – administered insulin passes to the regardless of weight and age of gestation.
placenta
- Baby is hypoglycaemic which leads to poor Landmarks in the History of Newborn Screening
mental performance 1961 Birth of Newborn Screening in US
- LGA/SGA 1996 24 MM Hospitals in the Philippines form the NBS Study Group
- Asphyxia 1999 DOH adopts NBS as a program
- Jitteriness/lethargy 2000 DOH AO No 1 s2000; creation of NTWG – NBS
- Hyperviscosity syndrome 2003 NBS Bill filed in congress and senate
o Hemoglobin/hematocrit 2004 Newborn Screening Act of 2004
- Prematurity 2008 1753 Newborn Facilities (NSF) in the Philippines
- Discordant twins
- Plethora or hyperviscosity What disorders are tested in newborn screening?
- Hypovolemia/hypotension 1. Congenital Hypothyroidism (CH)
- Fetal or neonatal blood loss 2. Congenital Adrenal Hyperplasia (CAH)
- LGA/SGA 3. Phenylketonuria (PKU)
- Maternal diabetes 4. Galactosemia (GAL)
- Pathologic jaundice 5. Glucose 6 Phosphate Dehydrogenase Deficiency (G6PD Def.)
- Maternal bleeding 6. Maple Syrup Urine Disease (Branched Chain Ketoaciduria)
- Suspected sepsis
o Newborn Screening Why are these disorders included in the NBS panel?
- PKU 1. Local prevalence
 2. Reversible if treated on time  Spastic hypertonic cerebral palsy in 30%
3. Treatment is available  Seizures in 20%
 EEG abnormalities in 80%
 Light colored hair
 Eczematoid rash/intractable itching
Disorders Confirmed Prevalence Laboratory diagnosis
CH 378 1:3,548  Bacterial inhibition assay using Bacillus subtilils – no bacterial
CAH 171 1:7,842 growth means lack of phenylalanine so the baby is positive for
GAL 15 1:89,401 PKU
PKU 10 1:134,102 Treatment
G6PD DEF. 23,557 1:54  dietary restriction of phenylalanine
 frequent measurement of phenylalanine plasma levels
Total number of screened as of December 2008  special formula for PKU patients
4 tests 1,341,017 Prognosis
G6PD 1,264,122  Mental deficiency is prevented if treatment is started before 2
months of life.
Benefits of Newborn Screening
 Detection of silent metabolic conditions as early as the first CONGENITAL HYPOTHYROIDISM
week of life Causes
 Prevention of neonatal or infant death (i.e. CAH)  Thyroid dysgenesis (most common)
 Facilitates accurate diagnosis when symptoms are nonspecific  Thyroid dyshormogenesis
or non-existent  Hypothalamic/pituitary dysfunction
 Makes effective management of metabolic disorders feasible  Maternal antithyroid drug ingestion
 Sample collection is simple, safe, inexpensive and reliable  Maternal autoimmune disorders
 Gives affected infants a chance at a normal life  Immaturity
Clinical Manifestations
GALACTOSEMIA  < 5% are symptomatic in the newborn period
 Deficiency of galactose-1-phosphate uridyl transferase  Most will manifest by 4th month of life
 This enzyme catalyzes the formation of uridin diphosphate Clinical Manifestations in Newborn Period
(UDP)-galactose to glucose-1-phosphate  Prolonged jaundice
 Results in increased galactose-1-phosphate and galactitiol in  Constipation
the liver, brain, kidney and eyes  Lethargy
 Autosomal recessive  Poor feeding
Clinical Manifestations  Hypothermia
 Failure to thrive – baby is hungry all the time that’s why he  Described as “good babies”
stops from growing  Usually quiet
 Hepatomegaly – no detoxification  Seldom cries when wet
 Jaundice  Loses one IQ point everyday
 Cataracts – glucose is found in lenses of the eyes Long term undetected congenital hypothyroidism leads to CRETINISM:
 Mental retardation  Developmental delay
 Renal failure (Fanconi syndrome)  Hollow empty eyes which appear far apart
Laboratory Diagnosis  Swollen eyelids
 Enzyme assay in RBC, cord blood, fibroblasts  Narrow palpebral fissures
Treatment  Narrow/depressed nasal bridge
 Exclusion of galactose from the diet Laboratory Diagnosis
 Use casein hydrolysates and soy milk substitutes (vegetable-  Determination of TSH by immunofluorescence
based milk)  Anemia
Prognosis  Increase indirect bilirubin
 Good with strict dietary control  Delayed bone age
 Cataracts are reversible if treatment is started within 5  Decreased T4, increase TSH
months of life  Thyroid scan
 Hepatic and renal manifestations are reversible  Radioactive Iodine Uptake
Treatment
PHENYLKETONURIA L-thyroxine
 Deficiency of phenylalanine hydroxylase which catalyzes the Early detection and treatment are crucial!
conversion of phenylalanine to tyrosine (for skin color and
neurotransmitters) GLUCOSE 6 PHOSPHATE DEHYDROGENASE DEFICIENCY
 Increased phenylalanine and degradation products in tissues  Deficiency of Glucose-6-phosphate dehydrogenase
 Autosomal recessive  Most common among Filipinos
 Mild signs and symptoms
Clinical Manifestations  X-linked – only males are affected
 Mental retardation: 99% of untreated Pathophysiology
 Failure to thrive  Glucose is responsible for the production of glutathione which
 Light skin fights free radicals
 Musty smelling Severe hemolysis after exposure to:
  Antimalarials (quinine/primaquine) MAPLE SYRUP URINE DISEASE (Branched Chain Ketoaciduria)
 Infections  Inability to breakdown branched chain amino acids (leucine,
 Acidosis isoleucine, valine)
 Sulfonamines  Deficiency of a multienzyme complex called branched chain
 Nitrofurans ketoacid carboxylase
 Antipyretic/analgesics ASA  Results in accumulation of branched chain amino acid in
 Sulfones plasma, urine, and CSF
 Others (vit K, PAS, naphthalene balls, phenylhydrasine,  Leucine and 2-oxoisocaproic acid are the most toxic to the
chloramphenicol) brain
Clinical Manifestations  Autosomal recessive
 Jaundice  Several molecular phenotypes
 Anemia
 Hemolysis can happen in all age groups Classic MUSD
Treatment  0-2% activity
 PRBC transfusion  Overwhelming illness in the first few days of life
 Avoidance of triggering factors  Lethargy - coma – seizures
 Sweet odor resembling burnt sugar detected in urine,
CONGENITAL ADRENAL HYPERPLASIA sweat, hair, and cerumen
 Family autosomal recessive disorders of cortisol biosynthesis  Hypoglycemia
 Cortisol deficiency increases ACTH producing adrenocortical Intermittent
hyperplasia and overproduction of intermediate metabolites  Milder manifestations
 Signs and symptoms depend on the enzymatic step that is  Patients become very sick when stressed
deficient Intermediate
 Milder manifestations
Signs and Symptoms  15-25% activity of enzymes
 No weight gain Thiamine Responsive
 Irritability  Similar manifestations as intermediate type
 Vomiting  Biochemical abnormality corrected with intake of high dose of
 Acute shock thiamine
 Ambiguous genitalia in females E-3 Variant
 Masculinization in males  Lactic acidosis
 Hypoglycemia  Similar symptomatology to classic MSUD
 Decreased Na, increased K  More rapid CNS depression
Treatment Diagnosis
 Cortisone  Plasma amino acid analysis (increased branched chain amino
 Salt replacement acid)
Types of Congenital Adrenal Hyperplasia (CAH)  Urine organic analysis; increased ketoacids
1. 21 Hydroxylase Deficiency  Branched chain ketoacid decarboxylase enzyme activity
 Most common (>90% of cases) determination in fibroblasts, leukocytes or amniotic fluid cells
 75% are salt losing form (definitive diagnosis)
 Decreased aldosterone  Presumptive diagnosis: urine + 2,4 dinitrophenylhydrazine
 Decreased cortisol (DNPH) – yellow precipitate
 Increased androgens (virilization)
 Develops at 2 weeks of age
 Death occurs in days or weeks if without treatment
 Vomiting – metabolic acidosis
 Acute shock
 Manifests sepsis-like symptoms
 Large genitals in males and ambiguous genitalia in
females due to high production of testosterone
Treatment
 Glucocorticoid replacement
 Mineralocorticoid for salt wasting form
 Surgical management of ambiguous genitalia
2. 11 β Hydroxylase Deficiency
 5-8% of cases
 Normal aldosterone
 Increased corticotropin
 Increased androgens (virilization)
 Hypernatremia
Treatment
 +/- mineralocorticoids
 Glucocorticoid for hypertension