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


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.


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.


Birth Defects

An abnormality of structure, function, or metabolism may result in a physical or mental disability, may shorten life, or may be fatal


Classification of Birth Defects

Malformations present at birth Metabolic defects (body chemistry) Blood disorders Chromosomal abnormalities Perinatal injury


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


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


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


Inborn Errors of Metabolism

May not always manifest at birth Screening tests important for early detection


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


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


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


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


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


Common Congenital Anomalies

Cleft lip and palate Esophageal atresia Spina bifida Hydrocephalus Developmental dysplasia of the hip Clubfoot Patent ductus arteriosus Tetralogy of Fallot


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


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


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


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


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


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


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


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


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


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


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


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


Signs of Hypoglycemia

Lethargy Hypotonia Jitteriness Poor feeding Tachypnea

Apnea Sweating Shrill cry Low temperature Seizures


Respiratory Disorders

Respiratory distress syndrome Meconium aspiration syndrome Transient tachypnea of the newborn Persistent pulmonary hypertension of the

newborn Sepsis


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


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


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


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


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


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


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


Common Congenital Defects

Associated with uncontrolled hyperglycemia throughout pregnancy

Defects include Congenital heart defects Tracheoesophageal fistulas CNS anomalies


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


Effects on the Newborn’s Metabolism


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.


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.


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


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


Infants of Diabetic Mothers (cont.)

Potential complications Polycythemia Hyperbilirubinemia Hypocalcemia Respiratory distress Macrosomia Birth defects


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


Neonatal Sepsis (cont.)

Neonate may initially be seen with Poor feeding Vomiting Diarrhea Lethargy

May later show Cyanosis Jaundice Hypothermia


Prevention of Neonatal Sepsis

Starts prenatally with maternal screening Sterile technique maintained through delivery Prophylactic antibiotics to newborn’s eyes


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)


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


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


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


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


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


Review Key Points


Chapter 16 The Newborn at Risk: Acquired and Congenital Conditions Copyright © 2012, 2008 by...

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