novobiocin and Hyperbilirubinemia--Hereditary

Topics: Adult; Altitude; Bilirubin; Biological Transport; Erythrocyte Aging; Female; Hemolysis; Hepatitis B; Hexosyltransferases; Humans; Hyperbilirubinemia; Hyperbilirubinemia, Hereditary; Infant, Newborn; Jaundice; Liver; Novobiocin; Portacaval Shunt, Surgical; Pregnancy; Vitamin K

Topics: Bilirubin; Coloring Agents; Glucosyltransferases; Glucuronates; Glycine; Humans; Hyperbilirubinemia; Hyperbilirubinemia, Hereditary; Indocyanine Green; Indoles; Infant, Newborn; Jaundice; Jaundice, Chronic Idiopathic; Jaundice, Neonatal; Liver; Metabolic Diseases; Novobiocin; Serum Albumin

Reduction in caloric intake was associated with a greater absolute rise in the serum bilirubin concentration in patients with Gilbert's syndrome and partial hepatic bilirubin uridine diphosphate glucuronyltransferase (UDPG-T) dysfunction compared to patients with hemolytic unconjugated hyperbilirubinemia and normal subjects. Two patients with overt hemolysis but an exaggerated response to caloric deprivation had reduced UDPG-T activities comparable to Gilbert's syndrome. The UDPG-T activities in the other patients with hemolytic jaundice were normal. The combination of fasting and novobiocin in 2 normal subjects produced a greater increase in bilirubin level than either fasting or novobiocin alone. These data suggest that theunderlying UDPG-T dysfunction, rather than the prefasting level of unconjugated hyperbilirubinemia, is responsible for the diet-induced hyperbilirubinemia in Gilbert's syndrome. The diet test appears to differentiate Gilbert's syndrome from hemolytic jaundice as well as from normal subjects, irrespective of the initial serum bilirubin concentration.

Topics: Anemia, Hemolytic; Diet; Fasting; Gilbert Disease; Glucuronosyltransferase; Hemolysis; Humans; Hyperbilirubinemia; Hyperbilirubinemia, Hereditary; Liver; Novobiocin

Topics: Chronic Disease; Ethanol; Female; Humans; Hyperbilirubinemia, Hereditary; Jaundice; Middle Aged; Novobiocin; Sulfobromophthalein

Topics: Animals; Bilirubin; Cholestasis; Dietary Fats; Female; Hyperbilirubinemia, Hereditary; Male; Microscopy, Electron; Novobiocin; Rats