Neonatal Jaundice PDF

Neonatal jaundice NNJ Hyperbilirubinemia CHAPTER 8 P: 252 Pdf : 284 Lecture Objectives:  Hyperbilirubinemia: Imbalance of bilirubin production and elimination In order to clear from body must be:  Conjugated in liver  E...

Neonatal jaundice NNJ Hyperbilirubinemia CHAPTER 8 P: 252 Pdf : 284 Lecture Objectives:  Hyperbilirubinemia: Imbalance of bilirubin production and elimination In order to clear from body must be:  Conjugated in liver  Excreted in bile  Eliminated via urine and stool  definition hyperbilirubinemia :refers to an excessive level of accumulated bilirubin in the blood and is characterized by jaundice, or icterus, a yellowish discoloration of the skin and other organs. Hyperbilirubinemia is a common finding in the newborn and in most instances is relatively benign. However, in extreme cases, it can indicate a pathologic state.  Pathophysiology REMINDER  Bilirubin is one of the breakdown products of hemoglobin that results from red blood cell (RBC) destruction.  When RBCs are destroyed, the breakdown products are released into the circulation, where the hemoglobin splits into two fractions: heme and globin.  The globin (protein) portion is used by the body, and the heme portion is converted to unconjugated bilirubin, an insoluble substance bound to albumin.  In the liver the bilirubin is detached from the albumin molecule and, in the presence of the enzyme glucuronyl transferase, is conjugated with glucuronic acid to produce a highly soluble substance, conjugated bilirubin glucuronide, which is then excreted into the bile.  …………………….  In the intestine, bacterial action reduces the conjugated bilirubin to urobilinogen, the pigment that gives stool its characteristic color.  Most of the reduced bilirubin is excreted through the feces; a small amount is eliminated in the urine  Normally the body is able to maintain a balance between the destruction of RBCs and the use or excretion of by-products.  However, when developmental limitations or a pathologic process interferes with this balance, bilirubin accumulates in the tissues to produce jaundice  Possible causes of hyperbilirubinemia in the newborn are:  Physiologic (developmental) factors (e.g., prematurity)  An association with breastfeeding or breast milk  Excess production of bilirubin (e.g., hemolytic disease, biochemical defects, bruises)  Disturbed capacity of the liver to secrete conjugated bilirubin (e.g., enzyme deficiency, bile duct obstruction)  Combined overproduction and underexcretion (increased hemolytic process)  Some conditions or disease states (e.g.G6PD] , hypothyroidism, galactosemia, infant of a diabetic mother)  Genetic predisposition to increased production (e.g., Native Americans, Asians)  ,,,,,,,,,,,,,  Hyperbilirubinemia may result from increased: 1. unconjugated or 2. conjugated bilirubin.  The unconjugated form (Table 8-2) is the type most commonly seen in newborns.  The following discussion of hyperbilirubinemia is limited to unconjugated hyperbilirubinemia  reminder Unconjugated bilirubin/indirect /fat soluble Conjugated bilirubin/ direct/water soluble  PHYSIOLOGIC JAUNDICE Cause 1. Immature hepatic function plus 2. increased bilirubin load from red blood cell (RBC) hemolysis; 3. enterohepatic shunting Onset After 24 hr (preterm infants, prolonged) Peak 2nd to 5th day, depending on ethnic origin, method of feeding  Duration Declines on 5th to 7th day  Therapy  Increase frequency of feedings and avoid supplements.  Evaluate stooling pattern.  Monitor transcutaneous bilirubin (TcB) or total serum bilirubin (TSB) level.  Perform risk assessment (see Fig. 8-11).  Use phototherapy if bilirubin levels increase significantly or significant hemolysis is present  BREAST FEEDING ASSOCIATED JAUNDICE (EARLY ONSET) Cause Decreased milk intake related to fewer calories consumed by infant before mother’s milk is well established; enterohepatic shunting; less frequent stooling………….slide Onset : 3rd to 4th day Peak : 3rd to 5th day Duration: Variable Therapy  Breastfeed frequently (10-12 times/ day); avoid supplements such as water, dextrose water, or formula.  Evaluate stooling pattern; stimulate as needed.  Perform risk assessment (see Fig. 8-11).  Use phototherapy if bilirubin levels increase significantly or significant hemolysis is present.  If phototherapy is instituted, evaluate benefits and harm of temporarily discontinuing breastfeeding; additional assessments may be required.  Assist mother with maintaining milk supply; feed expressed milk as appropriate.  After discharge, follow up according to age of infant at discharge and hour-specific nomogram bilirubin level at discharge (see p. 317—256 n).  BREAST MILK JAUNDICE (LATE ONSET)  Cause Possible factors in breast milk that prevent bilirubin conjugation. Less frequent stooling  Onset : 4th day  Peak :10th to 15th day  Duration :May remain jaundiced for 3-12 wk or more  Therapy  Increase frequency of breastfeeding; use no supplementation such as glucose water; cessation of breastfeeding is not recommended.  Perform risk assessment (see Fig. 8-11).  Consider performing additional evaluations: glucose-6-phosphate dehydrogenase, direct and indirect serum bilirubin, family history, and others as necessary.  May include:** home phototherapy with a temporary (10-12 hr) **discontinuation of breastfeeding; a subsequent TSB may be drawn to evaluate a drop in serum levels.  Assist mother with maintenance of milk supply and reassurance regarding her milk supply and therapy.  Use formula supplements only at practitioner’s discretion  HEMOLYTIC DISEASE  Cause : 1. Blood antigen incompatibility causing hemolysis of large numbers of RBCs. 2. Functional inability of liver to conjugate and excrete excess bilirubin from hemolysis  onset: During first 24 hr (levels increase ≥5 mg/dl/day)  Peak: Variable  Duration :Depends on severity and treatment therapy  Monitor TcB or TSB level.  Perform risk assessment (see Fig. 8-11).  Postnatal—  Use phototherapy; administer tin-mesoporphyrin, administer intravenous immunoglobulin per protocol; if severe, perform exchange transfusion.  Prenatal—  Perform intrauterine transfusion (fetus).  Prevent sensitization (Rh incompatibility) of Rh-negative mother with Rh immune globulin (RhIg) administration.  If mother is breastfeeding, assist with maintenance and storage of milk; may bottle-feed expressed milk as appropriate to therapy. Minimize maternal-infant separation, and encourage contact as appropriate.  Complications  Unconjugated bilirubin is highly toxic to neurons; therefore, an infant with severe hyperbilirubinemia is at risk of developing bilirubin encephalopathy,:- a term that describes early varying degrees of acute symptoms of bilirubin toxicity resulting from the deposition of unconjugated bilirubin in brain cells.  Kernicterus, or bilirubin-induced neurologic dysfunction, describes:- the yellow staining of the brain cells and brain cell necrosis that results in chronic, permanent changes to the brain secondary to bilirubin deposition in the brain.  The damage occurs when the serum concentration reaches toxic levels, regardless of cause.  The exact level of serum bilirubin required to cause damage is not yet known.  When certain pathologic conditions exist in addition to elevated bilirubin levels, the infant has an increased permeability of the blood- brain barrier to unconjugated bilirubin and, thus, potential irreversible damage.  Factors that enhance the development of bilirubin encephalopathy include :  acidosis, lowered serum albumin levels,  intracranial infections such as meningitis, and abrupt fluctuations in blood pressure.  any condition that increases the metabolic demands for oxygen or glucose (e.g., fetal distress, hypoxia, hypothermia, or hypoglycemia)  The risk is increased in late preterm infants, infants born preterm, male infants, breastfeeding infants, and those who are discharged early from the birth hospital without adequate follow-up  The administration of hypertonic solutions such as glucose and sodium bicarbonate in acutely ill infants, which causes a sudden rise in serum osmolality  Kernicterus: * Bilirubin deposits typically in basal ganglia, hippocampus, substantia nigra, etc.  Acute Bilirubin Encephalopathy/Kernicterus (Prodromal signs).  The signs of bilirubin encephalopathy are those of CNS depression or excitation:  increasing lethargy  decreased activity  irritability,  poor feeding  hypotonia,  high-pitched cry,  temperature instability, although some infants, particularly very low- birth-weight infants, may be asymptomatic  Later these subtle findings are followed by : development of athetoid cerebral palsy, extrapyramidal symptoms, opisthotonos, dental enamel hyperplasia of the primary teeth motor delay, seizures sensorineural hearing deficits Long-term effects :  neurologic damage  cognitive impairment  attention deficit hyperactivity disorder  delayed or abnormal motor movement (especially ataxia or athetosis)  behavior disorders, perceptual problems, or sensorineural hearing loss Physiologic Jaundice  The most common evidence of hyperbilirubinemia is the relatively mild and self-limited physiologic jaundice, or icterus neonatorum.  physiologic jaundice is not associated with any pathologic process.  almost all newborns experience elevated serum bilirubin levels, only about half demonstrate observable signs of jaundice.  Mechanisms Involved in Physiologic Jaundice 1. On average, newborns produce twice as much bilirubin as do adults because of higher concentrations of circulating erythrocytes and a shorter life span of RBCs (only 70 to 90 days, in contrast to 120 days in older children and adults). 2. the liver’s ability to conjugate bilirubin is reduced because of limited production of glucuronyl transferase. 3. a lower plasma-binding capacity for bilirubin because of lower albumin concentrations than older children. 4. Normal changes in hepatic circulation following birth may contribute to excessive demands on liver function.  5 the relatively sterile and less motile newborn bowel is initially less effective in excreting urobilinogen 6 In the newborn intestine the enzyme β-glucuronidase is able to convert conjugated bilirubin into the unconjugated form, which is subsequently reabsorbed by the intestinal mucosa and transported to the liver. This process, known as enterohepatic circulation or enterohepatic shunting, is accentuated in the newborn and is believed to be a significant factor in physiologic jaundice. FEEDING :  Feeding stimulates peristalsis and produces more rapid passage of meconium, thus diminishing the amount of reabsorption of unconjugated bilirubin  Feeding introduces bacteria to aid in the reduction of bilirubin to urobilinogen.  Colostrum, a natural cathartic, facilitates meconium evacuation  Breastfeeding-associated jaundice (early-onset jaundice) may begin as early as 2 to 4 days of age.  The jaundice is related to the process of breastfeeding and probably results from decreased caloric and fluid intake by breastfed infants before the milk supply is well established,  since fasting is associated with: 1. decreased hepatic clearance of bilirubin (If the newborn does not ingest adequate calories, the formation of hepatic binding proteins diminishes, resulting in higher bilirubin levels.) 2. A decrease in milk (fluid) intake may result in decreased stooling, increased weight loss, and increased fatty acid formation, which may interfere indirectly with hepatic uptake of bilirubin and conjugation  breastfeeding-associated jaundice is most likely a result of enterohepatic shunting rather than an increase in new bilirubin formation or abnormal bilirubin conjugation.  Supplemental fluids such as glucose water or water do not enhance bilirubin excretion and may delay the excretion process.  The current philosophy is to encourage more frequent breastfeeding, and thus increase stooling, while monitoring the infant’s bilirubin levels with transcutaneous monitoring or serum level  begins around the fourth day.  Rising levels of bilirubin peak during the second week and gradually diminish.  Despite high levels of bilirubin that may persist for 3 to 12 weeks, these infants are well.  The jaundice may be caused by factors in the breast milk (pregnanediol, fatty acids, and β-glucuronidase) that either 1. inhibit the conjugation or 2. decrease the excretion of bilirubin  CLINICAL MANIFESTATIONS:  jaundice, the yellowish discoloration primarily of the sclera, nails, or skin. As a rule, jaundice that appears within the first 24 hours is caused by HDN, sepsis, or one of the maternally derived diseases such as diabetes mellitus or infections.  Jaundice that appears on the second or third day, peaks on the third to fifth day, and declines on the fifth to seventh day is usually the result of physiologic jaundice.  The intensity of the jaundice is not always related to the degree of hyperbilirubinemia; therefore, transcutaneous screening or serum bilirubin levels are necessary. Diagnostic Evaluation for NNJ A) Bilirubin level TSB  TSB - Normal values of unconjugated bilirubin are 0.2 to 1.4 mg/dl. In the newborn, levels must exceed 5 mg/ dl before jaundice (icterus) is observable.  evaluation of jaundice is not based solely on serum bilirubin levels, but also on the timing of the appearance of clinical jaundice; gestational age at birth; age in days since birth; family history, including maternal Rh factor; evidence of hemolysis; feeding method; infant’s physiologic status; and progression of serial serum bilirubin levels.  B) RISK ASSESSMENT  The following risk factors are associated with pathologic hyperbilirubinemia in term and late-preterm infants; further investigation is warranted as to the cause, although the exact cause may remain undetermined:-  Appearance of clinical jaundice within 24 hours of birth  Serum bilirubin level or transcutaneous bilirubin in the high- risk zone of the hour-specific nomogram (Fig. 8-11)  Blood group incompatibility with a positive Direct Coombs test  Hereditary hemolytic disease such as G6PD  Gestational age 35 to 36 weeks  East Asian or Asian-American race  Cephalhematoma or significant bruising  Exclusive breastfeeding, especially infants experiencing difficulty breastfeeding or significant weight loss  History of sibling with hyperbilirubinemia Risk factor for jaundice: JAUNDICE Jaundice within first 24 hrs of life A sibling who was jaundiced as neonate Unrecognized hemolysis, UDP glucoronyl tarnsferase deficiency Non-optimal sucking/nursing Deficiency of G6PD Infection, IDM, Immaturity Cephalhematoma /bruising, Central hematocrit ( > 65%) or polycythemia East Asian/North Indian  The use of hour-specific serum bilirubin levels to predict newborns at risk for rapidly rising levels is now considered the gold standard for monitoring healthy neonates more than 35 weeks of gestation before discharge from the hospital.  Using a nomogram (see Fig. 8-11, A) with three designated risk levels (high, intermediate, or low risk) of hour specific total serum bilirubin values assists in determining which newborns might need further evaluation before and after discharge  C) SCREENING  Noninvasive monitoring of bilirubin via cutaneous reflectance measurements (transcutaneous bilirubinometry, or TcB) allows for repetitive estimations of TSB and, when used correctly, may decrease the need for invasive monitoring.  With shorter maternity stays, the value of transcutaneous bilirubin measurements as a screening tool for evaluating the need for obtaining serum bilirubin levels or closely monitoring the infant has received considerable attention.  TcB monitors provide accurate measurements within 2 to 3 mg/dl in most neonatal populations at serum levels below 15 mg/dl.  Multiple readings over time at a consistent site (e.g., sternum, forehead) are more valuable than a single reading.  Once phototherapy has been initiated, TcB is no longer useful as a screening tool. D) Careful clinical assessment and monitoring  Thorough history:  Pregnancy and delivery history  Labor induction  General health status and infectious risk  Feeding method and feeding progress  Vital signs and ins/outs (hydration status)  Risk factors for isoimmunization  Family history and ethnicity (ie. G6PD, spherocytosis, etc.)  Physical exam:  Activity level, feeding ability, bruising/hematoma, plethora E) Jaundice/Icterus:  Newborn icterus notable once total bilirubin > 5-6 mg/dL (versus older children/adults once > 2 mg/dL)  Progresses cranially to caudally  CAUTION: Visual assessment is subjective, inaccurate, and dependent on observer experience!  F) Diagnostic studies  Imaging: Liver/GB ultrasound, HIDA scan (r/o biliary atresia)  TREATMENT QUALITY PATIENT OUTCOMES NEONATAL HYPERBILIRUBINEMIA  Total serum bilirubin level will be maintained below high-risk zone on the hour-specific bilirubin nomogram.  Treatment  primary goals in the treatment of hyperbilirubinemia are to prevent bilirubin encephalopathy and kernicterus, and, as in any blood group incompatibility, to reverse the hemolytic process 1. The main form of treatment involves the use of phototherapy. 2. Exchange transfusion is generally used for reducing dangerously high bilirubin levels that occur with hemolytic disease. 3. Encourage Feeding 4. Medication. 5. Monitor feeding, stooling , voiding pattern , & weight.  Medications…..  A. phenobarbital ---hemolytic disease and is most effective when given to the mother several days before delivery.  Phenobarbital promotes : 1. hepatic glucuronyl transferase synthesis, which increases bilirubin conjugation and hepatic clearance of the pigment in bile. 2. protein synthesis, which may increase albumin for more bilirubin binding sites.  B. Bilirubin production in the newborn can be decreased by inhibiting heme oxygenase—an enzyme needed for heme breakdown (to biliverdin)—  The use of heme-oxygenase inhibitors provides a preventive approach to hyperbilirubinemia.  Medications………..2  C. IVIG is effective in reducing bilirubin levels in infants with Rh isoimmunization and ABO incompatibility.  Studies have shown a decrease in hospital stay and duration of phototherapy when IVIG is administered either as single-dose or two-dose regimens in neonates with hemolytic disease  feeding Healthy late-preterm and full-term infants with jaundice may also benefit from early initiation of feedings and frequent breastfeeding. HOW: REMINDER 1. increased intestinal motility 2. decreased enterohepatic shunting, 3. normal bacterial flora in the bowel to effectively enhance the excretion of unconjugated bilirubin. Management of Indirect Hyperbilirubinemia: Indications for Phototherapy (Term/Near-Term Infants): * Bhutani curves (as seen in AAP recommendations and YNHH NBSCU Guidelines)  Management of Breastfeeding Jaundice prevention and management of early onset jaundice in breastfed infants include encouraging frequent breastfeeding, preferably every 1.5 to 2 hours; avoiding glucose water, formula, and water supplementation; and monitoring for early stooling. The infant’s weight, voiding, and stooling should be evaluated along with the breastfeeding pattern. Parents are taught to evaluate the number of voids and evidence of adequate breastfeeding after the infant is home and are encouraged to call the primary care practitioner if:  there are indications the infant is not feeding well,  is difficult to arouse for feedings, or  is not voiding and stooling adequately.  Nursing Care Management  routine physical assessment includes observing for evidence of jaundice at regular intervals.  Jaundice is most reliably assessed by observing the infant’s skin color from head to toe and the color of the sclerae and mucous membranes.  Applying direct pressure to the skin, especially over bony prominences such as the tip of the nose or the sternum, causes blanching and allows the yellow stain to be more pronounced.  The nurse should observe the infant in natural daylight for a true assessment of color.  Also, bilirubin (especially at high levels) is not uniformly distributed in skin  ,,,,,,,,,,,,,,,  The transcutaneous bilirubin meter is a useful screening device to detect neonatal jaundice in full-term infants. Because phototherapy reduces the accuracy of the instrument, its value is limited to assessments made before the initiation of phototherapy  A careful history from the parents may reveal significant familial patterns of hyperbilirubinemia (e.g., older siblings of the infant).  Other considerations in assessment include the family’s ethnic origin (e.g., higher incidence in Asian infants); type of delivery (e.g., induction of labor); and infant characteristics such as significant weight loss after birth, gestational age, sex, and bruising.  Assess the method and frequency of feeding as well as the infant’s apparent hydration status.  Parent teaching  Encourage parents to keep a log of number of feedings, wet diapers, and stools, especially if the mother is breastfeeding and this is her first newborn.  Prevention of physiologic and breastfeeding jaundice may be possible with early introduction of feedings and frequent nursing without water supplementation.  Make every effort to provide an optimum thermal environment to reduce metabolic needs. HDN HEMOLYTIC DISEASE OF THE NEWBORN  HEMOLYTIC DISEASE OF THE NEWBORN HDN Hyperbilirubinemia in the first 24 hours of life is most often the result of hemolytic disease of the newborn (HDN), an abnormally rapid rate of RBC destruction. Anemia caused by this destruction stimulates the production of RBCs, which in turn provides increasing numbers of cells for hemolysis. Major causes of increased erythrocyte destruction are--- isoimmunization (primarily RhD) and ABO incompatibility.  Blood Incompatibility The membranes of human blood cells contain a variety of antigens, also known as agglutinogens, substances capable of producing an immune response if recognized by the body as foreign. The reciprocal relationship between antigens on RBCs and antibodies in the plasma causes agglutination (clumping). antibodies in the plasma of one blood group (except the AB group, which contains no antibodies) produce agglutination when mixed with antigens of a different blood group. In the ABO blood group system the antibodies occur naturally. In the Rh system the person must be exposed to the Rh antigen before significant antibody formation takes place and causes a sensitivity response known as isoimmunization.  Rh Incompatibility (Isoimmunization) the terms Rh positive (presence of antigen) and Rh negative (absence of antigen) are used in this discussion. The presence or absence of the naturally occurring Rh factor determines the blood type. Difficulty may arise when the mother is Rh negative and the infant is Rh positive the maternal and fetal circulations are separate, there is evidence of a bidirectional trafficking of fetal RBCs and cell free DNA to the maternal circulation.  …………. More commonly, however, fetal RBCs enter into the maternal circulation at the time of delivery The mother’s natural defense mechanism responds to these alien cells by producing anti-Rh antibodies Under normal circumstances, this process of isoimmunization has no effect on the fetus during the first pregnancy with an Rh-positive fetus, since the initial sensitization to Rh antigens rarely occurs before the onset of labor. increased risk of fetal blood being transferred to the maternal circulation during placental separation, maternal antibody production is stimulated. During a subsequent pregnancy with an Rh-positive fetus, these previously formed maternal antibodies to Rh-positive blood cells enter the fetal circulation, where they attach to and destroy fetal erythrocytes  risks of isoimmunization Multiple gestations abruptio placentae placenta previa manual removal of the placenta and cesarean delivery These factors increase the incidence of transplacental hemorrhage and subsequent isoimmunization  ……………. the condition begins in utero, the fetus attempts to compensate for the progressive hemolysis by accelerating the rate of erythropoiesis. As a result, immature RBCs (erythroblasts) appear in the fetal circulation—hence the term erythroblastosis fetalis. The development of maternal sensitization to Rh-positive antigens exhibits wide variability. Sensitization may occur during the first pregnancy if the woman previously received an Rh-positive blood transfusion.  …………. No sensitization may occur in situations in which a strong placental barrier prevents transfer of fetal blood into the maternal circulation. Approximately 10% to 15% of sensitized mothers have no hemolytic reaction with no adverse effects on the fetus. some Rh-negative women, even though exposed to Rh-positive fetal blood, are immunologically unable to produce antibodies to the foreign antigen.  erythroblastosis fetalis In the most severe form of erythroblastosis fetalis (hydrops fetalis), the progressive hemolysis causes : 1. fetal hypoxia; 2. cardiac failure; 3. generalized edema (anasarca); 4. fluid effusions into the pericardial, pleural, and peritoneal spaces (hydrops). 5. The fetus may be delivered stillborn or in severe respiratory distress  How to help 1. Maternal Rh immunoglobulin (RhIg) administration 2. early intrauterine detection of fetal anemia by ultrasonography (i.e., serial Doppler assessment of the peak velocity in the fetal middle cerebral artery), 3. and subsequent treatment by fetal blood transfusions 4. or high-dose intravenous immunoglobulin (IVIG) have dramatically improved the outcome of affected fetuses.  HDN…….2 ABO Incompatibility  ABO Incompatibility Hemolytic disease can also occur when the major blood group antigens of the fetus are different from those of the mother. The major blood groups are A, B, AB, and O. The presence or absence of antibodies and antigens determines whether agglutination will occur. Antibodies in the plasma of one blood group (except the AB group, which contains no antibodies) will produce agglutination (clumping) when mixed with antigens of a different blood group.  What's happen Naturally occurring antibodies in the recipient’s blood cause agglutination of a donor’s RBCs. The agglutinated donor cells become trapped in peripheral blood vessels, where they hemolyze, releasing large amounts of bilirubin into the circulation The most common blood group incompatibility in the neonate is between a mother with O blood group and an infant with A or B blood group. Hemolysis due to anti-A is more common than for anti-B (see Table 8-3 for possible ABO incompatibilities). Naturally occurring anti-A or anti-B antibodies already present in the maternal circulation cross the placenta and attach to fetal RBCs, causing hemolysis. TABLE 8.3 Potential Maternal-Fetal ABO Incompatibilities Maternal Blood Group Incompatible Fetal Blood Group O A or B B A or AB A B or AB  ………… Usually the hemolytic reaction is less severe than in Rh incompatibility; however, rare cases of hydrops have been reported. Unlike the Rh reaction, ABO incompatibility may occur in the first pregnancy. The risk of significant hemolysis in subsequent pregnancies is higher when the first pregnancy is complicated by ABO incompatibility  Clinical Manifestations Jaundice usually appears during the first 24 hours after birth, and serum levels of unconjugated bilirubin rise rapidly. Anemia results from the hemolysis of large numbers of erythrocytes, and hyperbilirubinemia and jaundice result from the liver’s inability to conjugate and excrete the excess bilirubin. Most newborns with HDN are not jaundiced at birth. However, hepatosplenomegaly and varying degrees of hydrops may be evident. If the infant is severely affected, hydrops, anemia, and hypovolemic shock are apparent. Hypoglycemia may occur as a result of pancreatic cell hyperplasia  Diagnostic Evaluation 1. A maternal antibody titer (indirect Coombs test) should be drawn at the first prenatal visit. 2. Genetic testing allows early identification of paternal zygosity at the Rh gene locus, thus allowing earlier detection of the potential for isoimmunization and avoiding further maternal or fetal testing 3. Amniocentesis can be used to test the fetal blood type of a woman whose antibody screen is positive; the use of polymerase chain reaction may determine the fetal blood type, hemoglobin, hematocrit, and presence of maternal antibodies. 4. The detection of cell-free fetal DNA in the maternal plasma of Rh-negative women to detect antigens an Rh-positive fetus has been used successfully. Such testing usually negates the necessity for amniocentesis for fetal blood type  ………….dx 5. US is an important adjunct in the detection of isoimmunization. Alterations in the placenta, umbilical cord, and amniotic fluid volume, as well as the presence of fetal hydrops, can be detected with high-resolution ultrasonography, allowing early, noninvasive treatment before the development of erythroblastosis. Serial Doppler US of fetal middle cerebral artery peak velocity is now the gold standard to detect and measure fetal hemoglobin and, subsequently, fetal anemia  Dx ……….. 6. Erythroblastosis fetalis caused by Rh incompatibility can also be assessed by evaluating rising anti-Rh antibody titers in the maternal circulation (indirect Coombs test) or by testing the optical density of amniotic fluid (delta OD 450 test), because bilirubin discolors the fluid. 7.FOR THE BABY (after birth): 1. The condition in the newborn is suspected on the basis of the timing and appearance of jaundice and confirmed postnatally by detecting antibodies attached to the circulating erythrocytes of affected infants (direct Coombs test or direct antiglobulin test). The Coombs test may be performed on umbilical cord blood samples from infants born to Rh-negative mothers if there is a history of incompatibility or if further investigation is warranted 2. RETIC COUNT , CBC, RH, BG,TSB ABO Incompatibility ……………….. 1. early detection and implementation of phototherapy for the reduction of hyperbilirubinemia. 2. The initial diagnosis is often more difficult because the direct Coombs test may be negative or weakly reactive. 3. presence of jaundice within the first 24 hours, elevated serum bilirubin levels, RBC spherocytosis, and increased erythrocyte production is diagnostic of ABO incompatibility. 4. In some centers IVIG transfusions are used in combination with phototherapy to treat ABO incompatibility 5. Exchange transfusion is not commonly required for ABO incompatibility except when phototherapy fails to decrease bilirubin concentrations.  Therapeutic Management The primary aim of therapeutic management of isoimmunization is prevention. Postnatal therapy usually entails phototherapy for mild cases and exchange transfusion for more severe forms. In severe cases of hydrops, aggressive interventions such as pericardial and pleural fluid aspiration, mechanical ventilatory support, and inotrope therapy may be required for stabilization. the hemolytic process may continue, causing severe anemia (if untreated) between 7 and 21 days of life  Prevention of Rh Isoimmunization A) RhIg (such as RhoGAM) must be administered to unsensitized mothers within 72 hours (but possibly as long as 3 to 4 weeks) after the first delivery, miscarriage, or abortion, and repeated after subsequent pregnancies. The administration of RhIg at 26 to 28 weeks of gestation further reduces the risk of Rh isoimmunization RhIg is not effective against existing Rh-positive antibodies in the maternal circulation. RhIg is administered intramuscularly, not intravenously, and only to Rh-negative women with a negative Coombs test—never to the infant. The injected anti-Rh antibodies destroy (by subsequent phagocytosis and agglutination) fetal RBCs passing into the maternal circulation before the mother’s immune system can recognize them B) Neonatal IVIG therapy neonatal IVIG therapy decreases the severity of RBC destruction (hemolysis) in HDN and subsequent development of neonatal jaundice. IVIG is believed to attack the maternal cells that destroy neonatal RBCs, slowing the progression of bilirubin production. C) The use of a heme-oxygenase inhibitor such as tin mesoporphyrin (administered IM to the newborn) has proved effective in treating hyperbilirubinemia in newborns with hemolytic disease D) Maternal administration of high-dose IVIG, alone or in combination with plasmapheresis, decreases the fetal effects of Rh isoimmunization  Intrauterine Transfusion consists of infusing blood into the umbilical vein of the fetus. The need for therapy is based on the antenatal diagnosis of fetal anemia by serial Doppler assessments of middle cerebral artery peak systolic velocity With the advance of US technology, fetal transfusion may be accomplished directly via the umbilical vein, infusing type O Rh-negative packed RBCs to raise the fetal hematocrit to 40% to 50%;  Exchange transfusion – For exchange transfusion, fresh whole blood is typed and cross-matched to the mother’s serum. CMV – negative ; whole blood less than 48 hours should be used is a standard mode of therapy for treatment of severe hyperbilirubinemia that is unresponsive to phototherapy or other therapies such as tin- mesoporphyrin The amount of donor blood used is usually double the infant’s blood volume, which is approximately 85 ml/kg body weight. The double-volume exchange transfusion replaces approximately 85% of the neonate’s blood. Baby kept NPO during exchange Exchange takes about 2 hours  ……………. Exchange transfusion : 1. removes the sensitized erythrocytes, 2. lowers the serum bilirubin level to prevent bilirubin encephalopathy, 3. corrects the anemia, and 4. prevents cardiac failure. Indications for exchange transfusion include rapidly increasing serum bilirubin levels and hemolysis despite aggressive phototherapy An exchange transfusion is a sterile surgical procedure. A catheter is inserted into the umbilical vein and threaded into the inferior vena cava. Management of Indirect Hyperbilirubinemia….. Indications for Exchange Transfusion (Term/Near-Term Infants): * Adapted from AAP recommendations and YNHH NBSCU Guidelines Risk and complications of exchange: Cardiac and respiratory disturbance. Shock due to bleeding or inadequate replacement of blood. Hyperkalemia , hypomagnesaemia , hypoglycemia , hyperglycemia. hypocalcemia Infection& necrotizing enterocolitis. Clot formation , air embolism, portal hypertension Procedure for exchange transfusion Perform by trained personal in NICU with monitoring & resuscitation capabilities. Observe neonate with a cardiopulmonary monitor & assess vital sings frequently…………monitor UVC is placed Laboratory studies : i. On initial blood :CBC, TSB, calcium level , blood cx ii. During procedure : calcium if sing of hypocalcemia is observed. iii. On final aliquot removed: CBC, TSB, calcium level  ……………. Accurate recording of blood volume exchange. Observe sings of hypocalcemia ( irritability, tachycardia , & prolonged QT interval).Calcium gluconate 10% is used to treat it at dose 1-2ml/kg. Evaluate medications. E.g. ampicillin , gentamycin ,digoxin , phenobarbiturate , vancomycin should be administered after exchange. Warm blood during exchange Shake blood bag q 15 min during exchange  ,,,,,,,,,,, If signs of cardiac or respiratory problems occur, the procedure is stopped temporarily and resumed once the infant’s cardiorespiratory function stabilizes. The nurse also observes for signs of transfusion reaction (e.g., temperature instability, hypotension, tachycardia, bradycardia, rash) maintains adequate neonatal thermoregulation, blood glucose levels, and fluid balance post exchange care: i continue phototherapy ii. Laboratory studies: TSB every 4 hours , serum glucose. iii. Monitor complications  ,,,,,,,,,,, If signs of cardiac or respiratory problems occur, the procedure is stopped temporarily and resumed once the infant’s cardiorespiratory function stabilizes. The nurse also observes for signs of transfusion reaction (e.g., temperature instability, hypotension, tachycardia, bradycardia, rash) maintains adequate neonatal thermoregulation, blood glucose levels, and fluid balance post exchange care: i continue phototherapy ii. Laboratory studies: TSB every 4 hours , serum glucose. iii. Monitor complications  ,,,,,,,,,,, If signs of cardiac or respiratory problems occur, the procedure is stopped temporarily and resumed once the infant’s cardiorespiratory function stabilizes. The nurse also observes for signs of transfusion reaction (e.g., temperature instability, hypotension, tachycardia, bradycardia, rash) maintains adequate neonatal thermoregulation, blood glucose levels, and fluid balance post exchange care: i continue phototherapy ii. Laboratory studies: TSB every 4 hours , serum glucose. iii. Monitor complications