Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ Key Features ++ Kernicterus is an irreversible brain injury characterized by choreoathetoid cerebral palsy and hearing impairment Intelligence is probably normal but may be difficult to assess because of associated hearing, communication, and coordination problems Infants with visible jaundice on the first day of life or who develop excessive jaundice require the following: Feeding and elimination history Birth weight and percent weight change since birth Examination for sources of excessive heme breakdown Assessment of blood type, Coombs testing, complete blood cell count (CBC) with smear, serum albumin, and total serum bilirubin (TSB) G6PD test if jaundice is otherwise unexplained, and in African-American infants with severe jaundice Fractionated bilirubin level in infants who appear ill, those with prolonged jaundice, acholic stool, hepatosplenomegaly, or dark urine to evaluate for cholestasis ++ Clinical Findings ++ Extrapyramidal movement disorder (choreoathetoid cerebral palsy) Gaze abnormality, especially limitation of upward gaze Auditory disturbances (deafness, failed auditory brainstem evoked response with normal evoked otoacoustic emissions, auditory neuropathy, auditory dyssynchrony) Dysplasia of the enamel of the deciduous teeth ++ Diagnosis ++ Clinical but is strengthened if audiologic testing shows auditory neuropathy and auditory dyssynchrony in which the otoacoustic emission test is normal but the auditory brainstem response is absent Magnetic resonance imaging (MRI) scanning of the brain is nearly diagnostic if it shows abnormalities isolated to the globus pallidus or the subthalamic nuclei, or both TSB should be measured and interpreted based on the age of the infant in hours at the time of sampling Term infants with a TSB level greater than the 95th percentile for age in hours have a 40% risk of developing significant hyperbilirubinemia ++ Treatment ++ Phototherapy Most common treatment for indirect hyperbilirubinemia Intensive phototherapy should decrease TSB by 30–40% in the first 24 hours, most significantly in the first 4–6 hours Infant's eyes should be shielded to prevent retinal damage Diarrhea, which sometimes occurs during phototherapy, can be treated if necessary by feeding a nonlactose-containing formula Exchange transfusion Double-volume exchange transfusion (~160–200 mL/kg body weight) is most often required in infants with extreme hyperbilirubinemia secondary to Rh isoimmunization, ABO incompatibility, or hereditary spherocytosis Also indicated in any infant with TSB above 30 mg/dL, in infants with signs of encephalopathy, or when intensive phototherapy has not lowered TSB by at least 0.5 mg/dL/h after 4 hours. The decision to perform exchange transfusion should be based on TSB, not on the indirect fraction of bilirubin. Procedure decreases serum bilirubin acutely by approximately 50% and removes about 80% of sensitized or abnormal red blood cells and offending antibody so that ongoing hemolysis is decreased Procedure is invasive, potentially risky, and infrequently performed GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options