Chapter 36 - Hemolytic Disorders and Congenital Anomalies Flashcards
Complications That Affect Newborns
Problems of gestational age or intrauterine growth that do not follow normal patterns
Acquired problems resulting from maternal or newborn physiologic factors
Physical problems
Hyperbilirubinemia
(lyzed RBC - goes to liver and baby’s liver can’t process so baby yellow)
Bilirubin level in blood is increased
Breakdown of hemoglobin formed after it is released from hemolyzed RBC’s
Characterized by yellow discoloration of the skin, mucous membranes, sclera, and various organs
Referred to as jaundice or icterus
Caused by an accumulation of unconjugated bilirubin and hemolyzed RBCs under the skin
Pathologic jaundice (hyperbilirubinemia)
Level of serum bilirubin that, if left untreated, can result in kernicterus (acute bilirubin encephalopathy)
IVF
Bili lights
Exchange transfusion
(heat in room 85 degrees, need baby to eat every 2 hours to get them to poop to get bilirubin out)
Acute Bilirubin Encephalopathy
Caused by deposition of bilirubin in brain
***Normally bilirubin does not damage-bound to albumin-carried to liver to become conjugated-if albumin binding sites are full circulates as unconjugated (indirect) bilirubin-highly lipid soluble and crosses blood brain barrier
Can develop in newborns who show no apparent signs of clinical jaundice
Bili 25 or >
Associated with acute and long-term symptoms of neurologic damage
Never present at birth
Kernicterus-irreversible-chronic sequela of bilirubin toxicity
ABO incompatibility
Fetal blood type is A, B, or AB, and the maternal type is O
Naturally occurring anti-A and anti-B antibodies are transferred across the placenta to the fetus
Maternal antibodies cross placenta, causing hemolysis of fetal RBC’s
Exchange transfusions required occasionally
Rh incompatibility (isoimmunization)
Intrauterine transfusion-can do as frequently as q 2 weeks into umbilical vein
Rh-positive offspring (inherited Rh positive gene from father) of an Rh-negative mother are at risk
- Mother forms antibodies against the fetal blood cells
Erythroblastosis fetalis-fetus compensates for by producing a large # of immature erythrocytes
Hydrops fetalis-most severe form of
(babies producing RBC by 9 wks of gestation)
Care Management of Hyperbilirubinemia
Determine blood type of woman prenatally
Rh immunoglobulin
Rhogam (RHoD) immunoglobulin-destroys fetal RBC’s in maternal circulation and blocks maternal antibody production
90% effective-give at Rh – mom 28 weeks,72 hours after delivery, after invasive procedure
Case Management of Hyperbilirubinemia
Inderect Coomb’s test-First prenatal visit of RH – woman, determines if mom has antibodies to Rh antigen
Cord blood to lab-indirect Coomb’s test antibody titer performed if titer is 1:64 exchange transfusion
Congenital Anomalies
Congenital disorder present at birth; can be caused by genetic or environmental factors, or both
Physical, metabolic, anatomic, or behavioral deviations from the normal pattern of development
Presence of one anomaly warrants need to evaluate for further anomalies
Most common major congenital anomalies that cause serious problems in neonate
Congenital heart disease Neural tube defects Cleft lip or palate Clubfoot Developmental dysplasia of the hip
Most common of all congenital anomalies
Cardiovascular system anomalies
81 per 10,000 births
Congenital heart defects (CHDs)
are anatomic abnormalities in the heart that are present at birth but may not be diagnosed immediately
***VSD most common 30-35% of all CHD (hole in ventricle)
Neural Tube Defects (NTD)
Defects in the closure of the neural tube during fetal development
Environmental influences: maternal anticonvulsants, MTX, folic acid deficiency, maternal diabetes
–> Folic acid supplement 0.4 mg/day (400 g to prevent NTD)
NTD 1:1000 births
Encephalocele-herniation of the brain and meninges through a skull defect
Anacephaly-absence of both cerebral hemispheres and of the overlying skull: incompatible with life
Central nervous system anomalies (neural tube defects)
Encephalocele Anencephaly Spina bifida Meningocele; myelomeningocele Hydrocephalus Microcephaly
Spina Bifida
Spina bifida-most common defect of CNS
Spina bifida occulta-milder form-skin covering defect-bulging-hairy patch
Spina bifida manifesta-herniation of the meninges
Myelomeningocele-herniation of the meninges and spinal cord-with or without skin covering-
Hydrocephalus
Excessive CSF in ventricles Usually obstruction not letting ventricles drain Increased head circumfrence Fontanels full Increasing ICP-vomiting, irritability
Microcephaly
HC 2 standard deviations below mean
Decreased brain growth
Respiratory system anomalies
Laryngeal web (uncommon) - Incomplete separation of two sides of the larynx-usually between vocal cords Choanal atresia (obligate nose breathers) - Posterior nares are blocked Congenital diaphragmatic hernia - Defect in the formation of the diaphragm-high mortality rate
Gastrointestinal system anomalies
Facial clefts—lip or palate Esophageal atresia and tracheoesophageal fistula Omphalocele Gastroschisis Gastrointestinal obstruction Imperforate anus
Cleft lip and palate
Among most common
Cleft lip with or without cleft palate 10.48 per 10,000 births
Cleft palate alone 6.39 per 10,000 births
Surgical repair-sometimes several surgeries
Feeding problems, risk of aspiration
Speech therapy
Orhodontics
(can have feeding problems, usually have special bottle, sometimes breast feeding works better)
Esophageal Atresia and TEF
EA-esophogus end in blind pouch
Tracheoesophagael fistula (TEF) abnormal connection between the esophagus and trachea
Surgically repaired
Can be seen on US
Omphalocele and Gastroschisis
Omphalocele-covered defect of umbilical ring, organs can herniate-50% have underlying chromosomal disorder
Gastorschisis-herniation of bowel through defect in abdominal wall to the right of the umbilical cord-no membranes covering
(have to have c-section; go to surgery soon after birth; short gut syndrome; will be on TPN - will kill liver so have to have liver and bowel transplant)
Imperforate Anus
1:5000 births Occur greater in male than female No anal opening Sometimes fistula (can get fixed then fine)
Musculoskeletal system anomalies
Developmental dysplasia of the hip (DDH)-abnormal development of one or all the components of the hip joint
Breech higher risk
Check for with Ortolani and Barlow tests
Clubfoot-severel variants-due to how foot can be positioned
Polydactyly (mult digits)
(have to be in harness to hold hips)
Genitourinary system anomalies
Hypospadias-abnormally located urinary meatus, anywhere along the ventral surface of the penis
Exstrophy of the bladder-rare, cover with sterile,nonadherent dressing-surgery within 48 hours
Ambiguous genitalia
Key Points
Hyperbilirubinemia caused by variety of factors, including maternal-fetal Rh and ABO incompatibility
Key Points
Erythroblastosis fetalis leads to anemia, edema, and the cytotoxic effects of unconjugated bilirubin
Key Points
Injection of Rho(D) immunoglobulin in Rh-negative and Coombs’ test–negative women bestows passive immunity and minimizes possibility of isoimmunization
Key Points
Neonatal exchange transfusion with type O, Rh-negative RBCs serves to treat anemia and acidosis and to remove bilirubin, maternal antibodies, and fetal RBCs that are beginning to hemolyze
Key Points
Major congenital defects are leading cause of death in term neonates
Key Points
Most common major congenital anomalies causing serious problems in the neonate Congenital heart disease Neural tube defects Cleft lip or palate Clubfoot Developmental dysplasia of the hip
Key Points
Minor anomalies may be part of a characteristic pattern of malformations
Key Points
Current technology permits the prenatal diagnosis of many congenital anomalies and disorders
Key Points
Most widespread use of postnatal testing for genetic disease is routine screening of newborns for inborn errors of metabolism
Key Points
Curative and rehabilitative problems of an infant with a congenital disorder are often complex and require a multidisciplinary approach to care
Key Points
Parents need special instruction (CPR, oxygen therapy, nutrition requirements) before taking home a high risk infant
Key Points
Supportive care given to parents of infants with an abnormal condition must begin at birth or at time of diagnosis and continue for years
