novobiocin and Anemia--Hemolytic

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: Analgesics; Analgesics, Non-Narcotic; Anemia; Anemia, Hemolytic; Antimalarials; Antipyretics; Erythroblastosis, Fetal; Female; Fetal Diseases; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Hyperbilirubinemia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Jaundice; Jaundice, Neonatal; Maternal-Fetal Exchange; North Carolina; Novobiocin; Pregnancy; Salicylates; Sulfisoxazole; Toxicology; Vitamin K

Topics: Anemia; Anemia, Hemolytic; Child; Hemolysis; Novobiocin; Toxicology