Kernicterus - Kernicterus Developmental Pediatrics Louis...

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Kernicterus Developmental Pediatrics Louis Meng, PL2 November 12 th 2002
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History Earliest work on jaundice from Baumes- 1785, and Hervieux-1847 Kernicterus was first described by Johannes Orth, 1875 He postulated that jaundice might have hematologic origins He noted that the brain in jaundiced adults wasn’t affected Christian Schmorl coined the term in 1904 Translated, Kernicterus means jaundice of the “kern” or nuclear region of the brain
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Pathophysiology RBCs are broken down Bilirubin is an end product of heme metabolism Bilirubin is conjugated in the liver Enzyme: UDP-Glucuronyl Transferase Conjugated bili is excreted via the GI tract Enzyme: Beta-Glucuronidase can unconjugate bili in the small intestine and bili is reabsorbed
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Bilirubin Conjugation
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Pathophysiology Newborn Hyperbilirubinemia Relatively high hematocrit; more cells to break down UDP-Glucuronyl Transferase is not fully functional until 3-4 months of life Relative starvation state and slow transit time, especially in breastfeeders Breastmilk contains beta-glucuronidase; enterohepatic circulation is increased
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Pathophysiology Exaggerated Hyperbilirubinemia Polycythemia Hemolysis Rh incompatibility ABO incompatibility Abnormal RBCs—G6PD, spherocytosis, thalassemia Birth Trauma—Bruising, Cephalohematoma Metabolic Abnormalities—Crigler Najjar, Gilbert Syndrome, Galactosemia Medications—Sulfonamides Displaces bilirubin from albumin; same binding site
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Pathophysiology UCB is lipophilic and crosses the Blood-Brain Barrier In vitro, free UCB will not precipitate out of solution unless in the presence of a polar lipid membrane In theory, only free UCB crosses, albumin-bound does not. BBB of infants is more permeable than adults, and acidosis causes it to be even more permeable. UCB has an affinity for the basal ganglia, hippocampus,
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Kernicterus - Kernicterus Developmental Pediatrics Louis...

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