Preventing Extreme Hyperbilirubinemia and Kernicterus
Most of the cases of kernicterus reported in the last decade did not occur in infants who had ABO or Rh hemolytic disease, but in apparently healthy term and near-term newborns with extremely high bilirubin levels (usually well above 30 mg/dL [513 μmol/L]) (124,390,406), in infants with G6PD deficiency (124,133,328,329), and in very sick newborns with low bilirubin levels (43). If we can prevent extreme hyperbilirubinemia we should be able to prevent almost all cases of kernicterus, but jaundice is very common and extreme hyperbilirubinemia (30 mg/dL [513 μmol/L] or higher) is rare, occurring in only about 1 in 10,000 infants (203). To ensure that we do not miss these rare infants, however, we need to follow and measure serum bilirubin levels in many infants, and treat some infants with phototherapy who will never develop severe hyperbilirubinemia (139,140). Ip and associates have calculated that 5 to 10 infants with TSB levels between 15 and 20 mg/dL (257 to 342 μmol/L) receive phototherapy to prevent 1 infant from reaching a TSB of 20 mg/dL (342 μmol/L) (139,140).
Hospital Policies and Jaundiced Newborns
According to recommendation 2.2 of the AAP Clinical Practice Guideline (76), “clinicians should ensure that all infants are routinely monitored for the development of jaundice, and nurseries should have established protocols for the assessment of jaundice. Jaundice should be assessed whenever the infant’s vital signs are measured, but no less than every 8 to 12 hours.” Recommendation 2.2.1 states that “protocols for the assessment of jaundice should include the circumstances in which nursing staff can obtain a TcB or order a TSB measurement.” Nursing staff have always assumed the responsibility for monitoring infants for jaundice but, for the first time, hospitals are now asked to develop protocols under which nurses can obtain a TcB or TSB without a physician order. Thus a nurse who notices jaundice in an infant who is younger than 24 hours old, will obtain a TcB or TSB and notify the physician of the result. If a TcB measurement is used as a screening tool, TSB measurements can be obtained by nurses whenever the TcB measurement is above a certain bilirubin percentile (see Fig. 35-20 and Noninvasive Measurements of Bilirubin above).
Assessing the Risk of Severe Hyperbilirubinemia
Recommendation 5.1 of the AAP Clinical Practice Guideline (76) states: “Before discharge, every newborn should be assessed for the risk of developing severe hyperbilirubinemia, and all nurseries should establish protocols for assessing this risk. Such assessment is particularly important in infants who are discharged before age 72 hours.” The guideline continues, “The AAP recommends 2 clinical options used individually or in combination for the systematic assessment of risk: predischarge measurement of the bilirubin level using TSB or TcB and/or assessment of clinical risk factors” (76). This must be followed by appropriate evaluation, monitoring, surveillance, and followup to ensure that severe hyperbilirubinemia is identified early and treated appropriately.
Clinical Risk Factors
Table 35-26 lists the risk factors for the development of hyperbilirubinemia. Almost all of these factors can be identified readily without recourse to the laboratory but, because these risk factors are common and the risk of severe hyperbilirubinemia is small, individually, these factors are of limited use as predictors of severe hyperbilirubinemia. Nevertheless, if no risk factors are present, the risk of severe hyperbilirubinemia is extremely low, and the more risk factors that are present, the greater the risk of severe hyperbilirubinemia (206). Some factors, such as breast-feeding, gestation age below 38 weeks, and significant jaundice (need for phototherapy) in a previous sibling, seem to be particularly important. It is remarkable that almost every recently described case of kernicterus occurred in a breast-fed infant, even when the infant had underlying G6PD deficiency (124,133,328,390).
Decreasing Gestation: The “Near-term” Newborn
Decreasing gestation has been repeatedly identified as a very important contributor to hyperbilirubinemia (197,206,211, 239,240,241). We found that infants of 36 weeks or less gestation
are 13 times more likely than infants at 40 weeks’ gestation to be readmitted for severe jaundice (241). Infants at 38 weeks of gestation are considered term babies yet they are 4 times more likely to develop a TSB. ≥25 mg/dL (428 μmol/L) than those at 40 weeks (206). Although these near-term infants, at 35 to 38 weeks of gestation, are cared for in well-baby nurseries, they are much more likely to nurse ineffectively, receive fewer calories, and have a greater weight loss than their truly term counterparts. When combined with less effective hepatic clearance because of prematurity, it is not surprising that they often become more jaundiced.
are 13 times more likely than infants at 40 weeks’ gestation to be readmitted for severe jaundice (241). Infants at 38 weeks of gestation are considered term babies yet they are 4 times more likely to develop a TSB. ≥25 mg/dL (428 μmol/L) than those at 40 weeks (206). Although these near-term infants, at 35 to 38 weeks of gestation, are cared for in well-baby nurseries, they are much more likely to nurse ineffectively, receive fewer calories, and have a greater weight loss than their truly term counterparts. When combined with less effective hepatic clearance because of prematurity, it is not surprising that they often become more jaundiced.
TABLE 35-26 RISK FACTORS FOR DEVELOPMENT OF SEVERE HYPERBILIRUBINEMIA IN INFANTS ≥35 WEEKS’ GESTATION (IN APPROXIMATE ORDER OF IMPORTANCE) | ||||
---|---|---|---|---|
|