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Type: Artigo de periódico
Title: Persistent Neonatal Hyperbilirubinemia Resulting From Gilbert's Syndrome In Association With Rhd Hemolytic Disease
Author: Facchini F.P.
De Assis A.M.
Abstract: Objective: To report on an infrequent association of pathologies causing considerable increase in bilirubin production and a signifiant decrease in its excretion. Description: The third pregnancy of an RhD negative woman. Her first child was normal and delivered to term and did not receive Rhogam. The second pregnancy was problematic due to Rh isoimmunization. She delivered a child to term, who required three exchange transfusions, but died on the 8th day of life. The third child was delivered at term, ORh positive, direct Coombs positive and had stump bilirubin of 6.5 mg/dl and hematocrit at 44%. Five hours after birth the child was jaundiced. Phenobarbital and intensive phototherapy were introduced. Hyperbilirubinemia was soon controlled, but relapsed whenever phototherapy was discontinued. On the 10th day of life the child received a transfusion for significant anemia. As jaundice persisted to the 13th day, associated Gilbert syndrome was considered and DNA sequence analysis was requested. The test demonstrated a mutant homozygote genotype UDPT1A1[TA] 7TAA. Phototherapy remained necessary until the 17th day of life and she was discharged from hospital the following day, after bilirubinemia had been controlled. She returned for follow-up and exhibited normal growth and neurological development. Comments: This case demonstrates the significance of increased bilirubin production/decreased bilirubin excretion causing intense hyperbilirubinemias and, in the abnsence of vigorous treatment, kernicterus. The effectiveness of intense phototherapy has also been demonstrated, reducing the risks of more aggressive treatments such as exchange transfusion. It also provides evidence of the importance of bilirubinemia follow-up until complete resolution. Copyright © 2005 by Sociedade Brasileira de Pediatria.
Rights: aberto
Identifier DOI: 10.2223/JPED.1395
Date Issue: 2005
Appears in Collections:Unicamp - Artigos e Outros Documentos

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