Gilbert's syndrome--a frequent cause of unconjugated hyperbilirubinemia in children after orthotopic liver transplantation

Pediatric Transplantation
Simone KathemannPatrick Gerner

Abstract

Gilbert's syndrome is one major cause for unconjugated hyperbilirubinemia in healthy individuals with the prevalence being approximately 3.2-8.6%. It is caused by a mutation in the promoter region of the UGT1A1-gene with a prolonged TAA-repeat coding for the enzyme bilirubin UDP-glucuronosyltransferase (A(TA)(7) TAA allele). After OLT, Gilbert's disease of the transplanted liver can cause unconjugated hyperbilirubinemia. Therefore, we looked for the presence of A(TA)(7) TAA alleles in pediatric liver transplant recipients with unconjugated hyperbilirubinemia. Laboratory results of 106 pediatric liver transplant recipients (aged 0-17 yr) were evaluated for elevated total bilirubin over 2.0 mg/dL (conjugated bilirubin <30%). In these patients, DNA of the liver graft was extracted from paraffin-embedded liver biopsy samples formerly taken for diagnostic reasons. The DNA was analyzed for A(TA)(7) TAA alleles in the promoter region of the UGT1A1-gene. In 4 of 106 pediatric liver transplant recipients we found unconjugated hyperbilirubinemia with total bilirubin above 2.0 mg/dL (conjugated bilirubin <30%). The analysis of the promoter region of the UGT1A1-gene of the liver grafts showed three homozygous A(TA)(7) TAA alleles (homozygo...Continue Reading

References

Jul 31, 1987·Deutsche medizinische Wochenschrift·A SiegB Kommerell
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Mar 10, 2004·International Journal of Hematology·Annamaria NosariEnrica Morra
Jul 22, 2006·Transplantation·Junichi KanekoMasatoshi Makuuchi

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Citations

Sep 30, 2014·Environmental Research·Hongmin FanJianjun Zhang
Jun 25, 2020·Clinical Transplantation·Murat AkyildizYaman Tokat
Apr 5, 2013·Transplant International : Official Journal of the European Society for Organ Transplantation·Sang Bin HanJae Won Joh
Sep 20, 2017·Seminars in Cardiothoracic and Vascular Anesthesia·Anthony BonaviaDmitri Bezinover

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