title: Marked hyperbilirubinemia associated with the heme oxygenase-1 gene promoter microsatellite polymorphism in a boy with autoimmune hemolytic anemia.
abstract: Mild hyperbilirubinemia is a clinical feature of hemolysis. Here we describe a boy with marked elevation of serum bilirubin values (maximum: 70 mg/dL) during an acute episode of autoimmune hemolytic anemia, which returned to within the reference range after clinical improvement. The boy was a homozygous carrier of short alleles of the heme oxygenase-1 (HO-1) gene GT dinucleotide-repeat promoter polymorphism, which is associated with increased activity and inducibility of the heme-degrading enzyme HO-1, which catalyzes the production of bilirubin. In addition, heterozygosity of the uridine 5'-diphosphate-glucuronosyl-transferase 1A1 promoter polymorphism that is linked with Gilbert syndrome was found in this patient. Because bilirubin production plays a critical role during the neonatal period, the HO-1 promoter polymorphism may be an important genetic factor for the clinical outcome of neonatal hyperbilirubinemia.
authors.affiliation: Institute of Clinical Immunology and Transfusion Medicine, Justus Liebig University, Giessen, Germany. stephan.immenschuh@immunologie.med.uni-giessen.de
authors.forename: Stephan
authors.initials: S
authors.lastname: Immenschuh
authors.forename: Ying
authors.initials: Y
authors.lastname: Shan
authors.forename: Hartmut
authors.initials: H
authors.lastname: Kroll
authors.forename: Sentot
authors.initials: S
authors.lastname: Santoso
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Wössmann
authors.forename: Gregor
authors.initials: G
authors.lastname: Bein
authors.forename: Herbert L
authors.initials: HL
authors.lastname: Bonkovsky
mesh_terms: D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000188:drug therapy; D016572:Cyclosporine / Q000627:therapeutic use; D005838:Genotype; D051547:Heme Oxygenase-1 / Q000235:genetics*; D006801:Humans; D006932:Hyperbilirubinemia / Q000097:blood / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D018895:Microsatellite Repeats / Q000235:genetics*; D011110:Polymorphism, Genetic / Q000235:genetics*; D013156:Splenectomy; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine; D051547:Heme Oxygenase-1