direct hyperbilirubinemia newborn differential

Several factors specific to the neonate’s physiology contribute to physiologic hyperbilirubinemia: Causes of pathologic hyperbilirubinemia can be classified as due to (1) increased bilirubin load (i.e., pre-hepatic; either hemolytic or non-hemolytic processes), (2) impaired bilirubin conjugation (i.e., hepatic) or (3) impaired bilirubin excretion (i.e., post-hepatic). Although breastfed infants are at a higher risk for developing severe hyperbilirubinemia than are formula-fed infants, the known risks of acute bilirubin encephalopathy are very small when weighed against the substantial known benefits of breastfeeding [17][63]. It should also be noted that although a large number of studies have demonstrated an increased risk of severe hyperbilirubinemia with breastfeeding, one study [24] found that exclusive breastfeeding was associated with a lower incidence of hyperbilirubinemia. Bilirubin is a yellow waste product that is formed when old or worn out red blood cells are broken down (hemolysis). Females with greater proportions of their red cells affected have an increased risk of severe neonatal hyperbilirubinemia [38]; therefore, testing of both girls and boys who are at risk is advised [39]. Kernicterus refers to the longterm effects of bilirubin toxicity. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation [published correction appears in Pediatrics. Side effects of phototherapy include temperature instability, intestinal hypermotility, diarrhea, interference with maternal-infant interaction and, rarely, bronze discolouration of the skin [41]. Bilirubin is measured in the blood as total bilirubin and direct bilirubin. Reticulocyte count (>7 mg/dL) can indicate the presence of an ongoing hemolytic process in neonates Direct hyperbilirubinemia in the neonate is defined as a direct fraction more than 2 mg/dL • or more than 20%of the total bilirubin concentration is always pathologic. The most common causes of hyperbilirubinemia are “ HOT Liver ”: H emolysis, O bstruction, T umor, and Liver disease. Increased Level of Unconjugated (Indirect) Bilirubin – hepatitis, cirrhosis, neonatal hyperbilirubinemia, transfusion reaction All jaundiced infants, especially high-risk infants and those who are exclusively breastfed, should continue to be closely monitored until feeding and weight gain are established and the TSB concentration starts to fall. Dana C. Matthews, Bertil Glader, in Avery's Diseases of the Newborn (Ninth Edition), 2012. In infants whose TSB concentration is approaching the exchange transfusion threshold, the addition of a fibre optic blanket under the infant can increase the surface area illuminated, and the diaper should then be removed (or a phototherapy wavelength-transmitting diaper used instead). Key Words: 35 weeks’ gestation; Hyperbilirubinemia; Jaundice; Preterm newborn; Term newborn. If levels continue to rise without improvement from intensive phototherapy (defined as at least 30µW/cm2 per nm as measured at the baby’s skin below the center of the phototherapy, compared with lower intensity conventional phototherapy), exchange transfusion may be indicated (see below). Several risk factors have been identified for the development of severe hyperbilirubinemia in the newborn (Table 1). Breastfeeding should continue, with lactation support as necessary. Increased Level of Conjugated (Direct Bilirubin) – gallstones, extra hepatic duct obstruction, extensive liver metastases. Revised and updated by a new editorial team, the Sixth Edition of this text will remain the leading reference on the clinical care of the newborn. 1959. Chronic bilirubin encephalopathy – the clinical sequelae of acute encephalopathy with athetoid cerebral palsy with or without seizures, developmental delay, hearing deficit, oculomotor disturbances, dental dysplasia and mental deficiency. The collaborative perinatal project, examining 54,795 live births in the United States, was unable to find any consistent association between peak TSB concentrations below critical levels and IQ or other adverse outcomes [12]. Transcutaneous bilirubin measurement provides more accurate information than clinical assessment. Previous recommendations were to measure TSB concentration in all infants with clinical jaundice at any time in the first four days of life, and to measure TSB concentration in those who are not clinically jaundiced but have increased risk factors. Intravenous immunoglobulin (IVIG) reduces bilirubin concentrations in newborns with rhesus hemolytic disease and other immune hemolytic jaundice. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation [published correction appears in Pediatrics. This edition of the Manual of Neonatal Care has been completely updated and extensively revised to reflect the changes in fetal, perinatal, and neonatal care that have occurred since the sixth edition. The incidence of chronic encephalopathy is also uncertain, but it has been estimated to be approximately one in 100,000 [19][20]. A healthy adult usually has a total bilirubin of about 1.2 milligrams per deciliter (mg/dL) of blood and 0.3 mg/dL for direct bilirubin. This process aims to remove bilirubin in the serum, as well as partially hemolyzed and antibody-coated red blood cells. G6PD-deficient newborns may require intervention at a lower TSB concentration because they are more likely to progress to severe hyperbilirubinemia [42][43]. Appropriate amounts of blood should be taken and stored for tests such as those for red cell fragility, enzyme deficiency (G6PD or pyruvate kinase deficiency) and metabolic disorders, as well as for hemoglobin electrophoresis and chromosome analysis. Consideration of further therapy should commence and preparations for exchange transfusion may be indicated. Exchange transfusion is a procedure with substantial morbidity that should only be performed in centres with the appropriate expertise under supervision of an experienced neonatologist. They also have slow intestinal motility in the first few days as feeding becomes established, and this increases small amounts of bilirubin reuptake by the enterohepatic circulation. There are several limitations to TcB measurements [50]: they become unreliable after initiation of phototherapy [51], and they may be unreliable with changes in skin colour and thickness [52]. This manual is a comprehensive guide to differential diagnosis in surgery. Greater than 37 weeks’ gestation and DAT-negative, 35 to 37 6/7 weeks’ gestation or DAT-positive, 35 to 37 6/7 weeks’ gestation and DAT-positive, *Arrangements must be made for a timely (eg, within 24 h) re-evaluation of bilirubin by serum testing. Supplemental fluids should be administered, orally or by intravenous infusion, in infants receiving phototherapy who are at an elevated risk of progressing to exchange transfusion (recommendation grade A). All infants presenting with symptoms and signs of ABE should receive immediate exchange transfusion. G6PD assay can be considered if ethnicity or family history confers increase risk of G6PD deficiency (though an X-linked recessive disorder, females heterozygotes can have ~50% of their red blood cells deficient due to random X chromosome inactivation). In most of cases there is no specific underlying disorder (physiologic). Hyperbilirubinemia is very common and usually benign in the term newborn infant and the late preterm infant at 35 to 36 completed weeks’ gestation. The conjugated bilirubin fraction should be estimated in an infant with persistent jaundice (longer than two weeks) and/or hepatosplenomegaly [60]. Complications include air embolism, vasospasm, infarction, infection and death. In addition to universal measurement, all newborns should be clinically assessed for jaundice repeatedly within the first 24 h, and again, at a minimum, 24 h to 48 h later. Crigler-Najjar syndrome is a rare genetic disorder characterized by elevated levels of bilirubin in the blood (hyperbilirubinemia). In an infant presenting with jaundice, total and conjugated bilirubin should be measured through capillary or a venous blood sample. Only infants who are at higher risk for requiring exchange transfusion should receive supplemental fluids, either orally (formula) or intravenously (D10W). Increased Level of Unconjugated (Indirect) Bilirubin – hepatitis, cirrhosis, neonatal hyperbilirubinemia, transfusion reaction Because of the excessive red blood cell breakdown, hemoglobin levels may be low or normal and reticulocyte (immature erythrocyte) count may be elevated. Investigations should include a clinically pertinent history of the baby and the mother, family history, description of the labour and delivery, and the infant’s clinical course [35]. Rotor syndrome is diagnosed based on symptoms and various laboratory tests. Sudden increases in TSB concentration may also occasionally occur after the first two to three days [47]. It is important to remember that hyperbilirubinemia is not a disease, per se, but rather a characteristic of a disease. There are two forms of bilirubin in the body: a toxic form called unconjugated bilirubin and a nontoxic form called conjugated bilirubin. Thank you, DAT Direct antiglobulin test. For permission to reprint or reproduce multiple copies, please see our copyright policy. Found inside – Page 1099In any jaundiced infant, it is necessary to determine whether jaundice is due to conjugated versus unconjugated hyperbilirubinemia. Although the differential of potential causes will vary by the type of predominant bilirubin, ... A TSB concentration greater than 30 µmol/L in umbilical cord blood [29] is statistically correlated with a peak neonatal TSB concentration greater than 300 µmol/L, but the positive predictive value is only 4.8% for the term infant, rising to 10.9% in the late preterm infant, and the specificity is very poor (evidence level 1b). A newborn was noted to be cyanotic. Reports [22][23] indicate that acute bilirubin encephalopathy continues to occur in otherwise healthy infants with, and occasionally without, identifiable risk factors. Bilirubin is a yellow waste product that is formed when old or worn out red blood cells are broken down (hemolysis). In this scenario, transfer to a Level III Neonatal Intensive Care unit is indicated. Hemolytic disease of the fetus and newborn should be considered in the differential diagnosis of newborns with jaundice/hyperbilirubinemia and certainly in the case of neonatal anemia. Infants with a TSB concentration above the thresholds shown on. Extravascular hemolysis occurs in the spleen and liver, which sequesters damaged RBCs. Blood group and direct Coombs testing in babies who are at risk of Rh or ABO isoimmunization. Learn everything an expat should know about managing finances in Germany, including bank accounts, paying taxes, getting insurance and investing. Intensive phototherapy for infants with severe hyperbilirubinemia or those at greatly elevated risk of developing severe hyperbilirubinemia. Mechanism: Phototherapy involves exposing the skin to blue wavelengths of light. Interrupting breastfeeding is, however, associated with markedly reduced rates of breastfeeding continuation after 1 month. The peak TSB concentration usually occurs between three and five days of life, at which time the majority of babies have already been discharged from hospital. Phototherapy can be used both to prevent severe hyperbilirubinemia in infants with a moderately elevated TSB concentration and as initial therapy in those with severe hyperbilirubinemia. A full-size downloadable version of this graph. Neonatal jaundice is a yellowish discoloration of the white part of the eyes and skin in a newborn baby due to high bilirubin levels. Complications may include seizures, cerebral palsy, or kernicterus.. Carefully timed TSB measurements can be used to predict the chances of developing severe hyperbilirubinemia. Treatment should be stopped once total bilirubin is below the treatment threshold. G6PD deficiency increases the likelihood of requiring exchange transfusion in infants with severe hyperbilirubinemia; therefore, a test for G6PD deficiency should be considered in all infants with severe hyperbilirubinemia (evidence level 5). Learn everything an expat should know about managing finances in Germany, including bank accounts, paying taxes, getting insurance and investing. In general, fluorescent light is most commonly used [81]; the intensity of light produced by fluorescent tubes wanes over time. 2004;114(1):297–316. Breastfed babies are at higher risk of developing hyperbilirubinemia as compared to those that are formula-fed, however, the proven benefits of breastfeeding substantially outweigh the risks of hyperbilirubinemia, and thus should continue if possible. Although bilirubin is derived from the breakdown of hemoglobin, routine umbilical cord blood hemoglobin or hematocrit measurement does not aid in the prediction of severe hyperbilirubinemia [30] (evidence level 2b). Guidelines for the prediction, prevention, identification, monitoring and treatment of severe hyperbilirubinemia are presented. Pediatrics . Reassurance of the parents that appropriate intervention and follow-up will prevent any consequences of hyperbilirubinemia is an important part of the care of these infants. Learn how we are healing patients through science & compassion, Stanford team stimulates neurons to induce particular perceptions in mice's minds, Students from far and near begin medical studies at Stanford. Physical exams in affected people are typically normal, except for mild jaundice. Congratulations to my chairman Dr Vaughn Starnes 100th AATS…” Newborns with glucose-6-phosphate dehydrogenase (G6PD) deficiency have an increased incidence of severe hyperbilirubinemia (evidence level 1b). Total bilirubin levels increase with any type of jaundice; direct and indirect bilirubin … Phototherapy in the neonatal period is perceived by parents as implying that their infant’s jaundice was a serious disease [78], and is associated with increased anxiety and health care use (evidence level 2). [1] Approximately 80% of the bilirubin is derived from hemoglobin metabolism. This treatment is considered in infants with total bilirubin concentration between 375 umol/L and 425 umol/L, without response to intensive phototherapy, in the presence of severe anemia or hemolytic disease or rapid rise in total bilirubin (> 17umol/L in less than 6 hours). Found insideNeonatal hyperbilirubinemia is the result of increased production and/or a diminished ability to conjugase and excrete bilirubin. Newborn red blood cells have a short life span, and preterm infants are at even higher risk of jaundice.

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