Chapter 16 Integumentary Disorders Copyright © 2012 by Saunders, an imprint of Elsevier, Inc.
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Transcript of Chapter 16 The Newborn at Risk: Acquired and Congenital Conditions Copyright © 2012, 2008 by...
Chapter 16
The Newborn at Risk:
Acquired and Congenital Conditions
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 1
Chromosomal Disorders
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 2
Objectives
Define key terms listed. Discuss the prenatal diagnosis of Down
syndrome. Recognize three out of four genetic inborn
errors of metabolism. Compare the metabolic disorders
hypoglycemia, maple syrup urine disease, hypothyroidism, and phenylketonuria; their effect on the newborn; and the nursing implications.
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 3
Objectives (cont.)
Describe common congenital anomalies. Interpret signs associated with elevated
bilirubin in the newborn. Explain the nursing interventions used in
phototherapy. Articulate the principles of newborn
resuscitation. Outline the common respiratory problems in
the newborn.
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 4
Birth Defects
An abnormality of structure, function, or metabolism may result in a physical or mental disability, may shorten life, or may be fatal
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 5
Classification of Birth Defects
Malformations present at birth Metabolic defects (body chemistry) Blood disorders Chromosomal abnormalities Perinatal injury
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 6
Chromosomal Disorders
Abnormal chromosome number or arrangement can cause congenital defect
May receive too little or too much genetic material
As embryo develops, genetic information is “scrambled” and may translate into a variety of congenital defects
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 7
Down Syndrome
Trisomy 21 (extra chromosome) Most common chromosomal syndrome
Mothers older than 35 have higher incidence First trimester screening: ultrasound
assessment of thickness of fetal nuchal fold Second trimester screening: absence of nasal
bone or “quad test” of blood for alpha-fetoprotein (AFP), human chorionic gonadotropin (hCG), unconjugated estriol (UE), and inhibin A
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 8
Characteristics of Down Syndrome
Craniofacial abnormalities Straight simian crease in palm
of hand Mental retardation with IQ
around 50 Females are fertile; males are
usually infertile
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 9
Inborn Errors of Metabolism
May not always manifest at birth Screening tests important for early detection
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 10
Phenylketonuria (PKU)
Autosomal-recessive, inherited inborn error of phenylalanine Faulty metabolism of an amino acid essential to
life and found in all protein foods Hepatic enzyme phenylalanine hydrolase, which is
needed to convert phenylalanine to tyrosine, is missing
Once infant ingests proteins, phenylketones accumulate in blood and then in brain
Can result in pronounced brain damage
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 11
Phenylketonuria (PKU) (cont.)
Diagnosed by Guthrie test Best if blood obtained after infant has
ingested protein Treatment is with phenylalanine-restricted
diet Goals of diet are to provide essential proteins to
support growth and development and keep phenylalanine blood levels between 2 and 10 mg/dL
• Less than 2 mg/dL can cause growth retardation
• More than 10 mg/dL can cause brain damage
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 12
Galactosemia
Deficiency in enzyme needed to convert galactose to glucose
Can cause failure to thrive, cataracts, jaundice, cirrhosis of liver, sepsis, and mental retardation
Breastfeeding is contraindicated due to the lactose
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 13
Hypothyroidism
Caused by maternal iodine deficiency or use of antithyroid drugs
If not treated with thyroid replacement, hypothermia, poor feeding, lethargy, jaundice, and cretinism can occur
Infant has large protruding tongue, thick lips, and a dull appearance
Testing for hypothyroidism is mandated nationwide
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 14
Maple Syrup Urine Disease
The amino acids leucine, isoleucine, and valine cannot be metabolized due to missing enzymes Elevated levels of leucine cause cerebral edema
and CNS symptoms Body fluids have a sweet odor
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 15
Common Congenital Anomalies
Cleft lip and palate Esophageal atresia Spina bifida Hydrocephalus Developmental dysplasia of the hip Clubfoot Patent ductus arteriosus Tetralogy of Fallot
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 16
Hyperbilirubinemia (Physiologic Jaundice)
Physiologic jaundice; icterus neonatorum Metabolism and excretion of excess waste
products, liver immaturity, delayed feeding, trauma, or cold stress
Can also occur due to cephalohematoma, extensive bruising, infections, acidosis
Typically occurs third day of life and peaks around fifth day
Total serum bilirubin level over 12 mg/dL
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 17
Hyperbilirubinemia (Physiologic Jaundice) (cont.)
Skin and whites of eyes assume yellow-orange cast
The higher the bilirubin, the deeper the jaundice
Conjugation of bilirubin inhibited by lack of bacteria in intestines or low levels of glucuronyl transferase enzyme
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 18
Hyperbilirubinemia (Physiologic Jaundice) (cont.)
High levels of bilirubin can stain basal nuclei of brain and cause kernicterus
Stimulation of meconium stool passage Early feedings enhance passage Colostrum in breast milk has natural laxative effect
Glucose water avoided, since little bilirubin is excreted and can increase absorption of bilirubin due to retained stool
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 19
Assessment of Physiologic Jaundice
Blanch test Yellow tinge appears
Head-to-toe progression of jaundice The more of the body that is yellow-orange,
the higher the bilirubin level Serum test for blood levels Transcutaneous measuring via hand-held
device
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 20
Hyperbilirubinemia (Pathologic Jaundice)
Hemolytic disease – excessive breakdown of RBCs due to maternal antibodies passing through placenta to fetus
Isoimmune hemolytic disease (erythroblastosis fetalis) Rh– mother pregnant with Rh+ fetus Transplacental passage of maternal antibodies Fetal system responds by increasing RBCs, and
many are immature
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 21
Hyperbilirubinemia (Pathologic Jaundice) (cont.)
At birth, newborn with hemolytic disease caused by Rh incompatibility will have a positive direct Coombs’ test
Reveals presence of antibody-coated (sensitized) Rh-positive RBCs in newborn
Indirect Coombs’ test measures amount of Rh-positive antibodies in mother’s blood
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 22
Management of Jaundiced Newborn
Goal is prompt identification of newborns at risk for jaundice and early implementation of treatment to prevent development of kernicterus
Newborn hyperbilirubinemia considered pathologic if jaundice is evident within first 24 hours of life
Clinical jaundice persists for more than 14 days
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 23
Clinical Signs of Kernicterus
Temperature instability Poor feeding Decreased muscle
tone Poor Moro reflex Lethargy High-pitched cry Rigidity Irritability
Opisthotonos position
Seizures Upward gaze Dark urine Light stools Apnea and seizures
possible in preterm newborns
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 24
Phototherapy
Blue light spectrum decreases bilirubin levels
Converts unconjugated bilirubin into isomers called photobilirubin, which is transported to liver, where it combines with bile and is excreted in feces and some in urine
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 25
Blood Exchange Transfusion May be indicated if bilirubin level greater than
25 mg/dL Alternately, remove small amount of
newborn’s blood from umbilical vessels and replace with donor blood
Rh-negative blood is used Observe for transfusion reaction
Jitteriness Seizures Edema Signs of fluid overload
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 26
RhoGAM
Prevents erythroblastosis fetalis IM injection given to an Rh-negative mother
within 72 hours of delivery of an Rh-positive newborn, provided she has not been previously sensitized Can also be given at 28 weeks gestation
• Need RhoGAM also after an abortion, after amniocentesis, or if bleeding occurs during pregnancy
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 27
Hypoglycemia
Two consecutive low values on blood taken 30 minutes apart
Plasma glucose less than 25 mg/dL usually treated with IV glucose
Newborns of diabetic mothers at risk for hypoglycemia when insulin levels remain high and glucose from placenta is decreased
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 28
Signs of Hypoglycemia
Lethargy Hypotonia Jitteriness Poor feeding Tachypnea
Apnea Sweating Shrill cry Low temperature Seizures
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 29
Respiratory Disorders
Respiratory distress syndrome Meconium aspiration syndrome Transient tachypnea of the newborn Persistent pulmonary hypertension of the
newborn Sepsis
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 30
Respiratory Distress Syndrome (RDS)
Hyaline membrane disease Major cause of newborn morbidity and death Impaired or delayed surfactant key role Hypoxemia, metabolic acidosis, pulmonary
vasoconstriction Decreased ability to exchange oxygen and
carbon dioxide and remove metabolic waste products
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 31
Respiratory Distress Syndrome(RDS) (cont.)
Initially see tachypnea, nasal flaring, subcostal and intercostal retractions, cyanosis, and respiratory grunting
Signs usually appear within 1 hour of birth Silverman-Anderson index looks at
Chest and abdominal movement Intercostal spaces Xiphoid area Nares Expiratory sound
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 32
Respiratory Distress Syndrome (RDS) (cont.)
Avoid preterm birth If necessary, mother is given corticosteroids
to stimulate fetal lung production of surfactant After birth, administration of surfactants by
trachea to newborn
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 33
Meconium Aspiration Syndrome
Fetal physical response to asphyxia in utero, causing Increased intestinal peristalsis Relaxation of anal sphincter Passage of meconium into amniotic fluid
Fetus experiences hypoxia; gasping movements draw meconium into fetal airways
Rarely occurs in fetuses younger than 34 weeks gestation
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 34
Meconium Aspiration Syndrome (cont.)
Obstruction of large or upper airways leads to hypoxic emergency Meconium in lungs cause a ball-valve action Air is allowed in but cannot escape
Causes Overdistention of alveoli Leads to alveolar rupture Pulmonary air leaks Chemical inflammation Atelectasis
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 35
Persistent Pulmonary Hypertension of the Newborn
(PPHN) Combination of pulmonary hypertension and
persistence of right-to-left shunting in the heart Fetal circulation persists even after delivery (also
called persistent fetal circulation) Could be due to single entity or main
component of meconium aspiration syndrome, pneumonia, sepsis, or diaphragmatic hernia
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 36
Transient Tachypnea of the Newborn (TTN)
Seen more in cesarean births May be a result of insufficient thoracic squeeze Results in retained fetal lung fluid
Respiratory rate increases to help rid lungs of retained fluid Between 100 and 140 breaths/min
Will show expiratory grunting, nasal flaring, and mild cyanosis
Usually self-limiting
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 37
Common Congenital Defects
Associated with uncontrolled hyperglycemia throughout pregnancy
Defects include Congenital heart defects Tracheoesophageal fistulas CNS anomalies
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 38
Audience Response SystemQuestion 1
Decreased ability to exchange oxygen and carbon dioxide and remove metabolic waste products indicates the infant has what type of syndrome?
A.Hypothryoidism
B.Respiratory distress
C.Phenylketonuria
D.Maple syrup urine
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 39
Effects on the Newborn’s Metabolism
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 40
Objectives
Characterize the effect of maternal diabetes on the newborn.
Outline six problems of infants born to mothers with diabetes mellitus.
Explain factors responsible for newborn sepsis, and state the nurse’s role in reducing the risks.
Discuss the nursing assessment that would lead the nurse to suspect newborn sepsis.
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 41
Objectives (cont.)
Identify the defects involved in the tetralogy of Fallot and common manifestations.
Compare the alteration of blood flow of cyanotic and noncyanotic congenital heart defects.
Explain the pathophysiology of noncyanotic congenital heart defects.
Describe care of the newborn who has neonatal abstinence syndrome.
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 42
Infants of Diabetic Mothers
Long-term diabetic mothers May have deficient nutrients as a result of
decreased blood flow reaching fetus Hypertension can further compromise
uteroplacental blood flow Can cause fetal growth restriction and/or death
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 43
Infants of Diabetic Mothers (cont.)
Increased risk of respiratory distress syndrome High levels of insulin interfere with production of
surfactant Hypoglycemia after birth
Blood glucose less than 40 mg/dL in term Blood glucose less than 30 mg/dL in preterm
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 44
Infants of Diabetic Mothers (cont.)
Potential complications Polycythemia Hyperbilirubinemia Hypocalcemia Respiratory distress Macrosomia Birth defects
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 45
Neonatal Sepsis
Infection occurring within the first month after birth
Diagnosis is based on clinical presentation and positive blood cultures Most common organisms are Staphylococcus
aureus, Staphylococcus epidermidis, Escherichia coli, Haemophilus influenzae, and group B streptococci
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 46
Neonatal Sepsis (cont.)
Neonate may initially be seen with Poor feeding Vomiting Diarrhea Lethargy
May later show Cyanosis Jaundice Hypothermia
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 47
Prevention of Neonatal Sepsis
Starts prenatally with maternal screening Sterile technique maintained through delivery Prophylactic antibiotics to newborn’s eyes
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 48
Management of Neonatal Sepsis
Cultures of blood, urine, stool, spinal fluid, and, in some case, IV catheter tips
Cultures from areas with drainage (e.g., eyes and umbilical stump)
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 49
Newborn with Effects of Maternal Substance Abuse
Newborn may display various physical and neurobehavioral manifestations
May be small for gestational age or have congenital anomalies due to maternal substance abuse
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 50
Signs of Neonatal Abstinence Syndrome
Respiratory Distress
GI Dysfunction
CNS Other
Stuffy nose
Tachypnea
Flaring of nares
Retractions
Apnea
Diarrhea
Vomiting
Frantic sucking
Poor feeder
Shrill, high-pitched cry
Irritability
Hypertonicity
Tremors
Short sleep cycles
Occasional seizures
Sweating
Fever
Sneezing
Yawning
Mottled color
Abrasions of elbows and knees
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 51
The Nurse and the Family of the Newborn at Risk
Role of the nurse To provide support by helping parents recognize
reality of problem Establish trust in health care provider Dispel misconceptions Mobilize family support systems Help parents participate in newborn care where
possible
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 52
Discharge Planning and Home Care
Begins as soon as disorder or problem is identified
Multiple disciplines are involved in the discharge planning and teaching process
Requires parental competence with the various equipment, supplies, and care techniques that may be required
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 53
Audience Response SystemQuestion 2
Discharge to the home setting for a newborn at risk requires:A.Multidisciplinary team management
B.Parental competence
C.Approval from the insurance company
D.Support and encouragement
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 54
Review Key Points
Copyright © 2012, 2008 by Saunders, an imprint of Elsevier Inc. 55