Unusual early presentation of Gilbert syndrome in pediatric recipients of liver transplantation

Background: Gilbert syndrome as a rule becomes manifest in adolescence or i n early adulthood, it may be transferred by the donor to orthotopic liver t ransplant (OLT) recipients. Methods: We examined the frequency of Gilbert syndrome in 46 OLT pediatric recipients who had a follow-up of I year or more. Diagnostic criteria inclu ded unexplained chronic or re current unconjugated hyperbilirubinemia; its increase after reduced caloric intake plus prolonged fasting, without chang es of the proportion of conjugated bilirubin; and high relative amounts of serum unconjugated bilirubin IXa and prevalence of the monoglucuronide over the diglucuronide. Results: Of the 46 patients, 42 had normal bilirubin values. Only four othe rwise healthy OLT recipients showed hyperbilirubinemia and normal conjugate d fractions. Liver donors had been four men. Hyperbilirubinemia persisted w ith a fluctuating pattern for the whole follow-up after OLT in all. Total b ilirubin level in blood samples obtained after reduced caloric intake and p rolonged fasting became notably higher than basal values, whereas the propo rtion of conjugated bilirubin remained stable. High relative amounts of unc onjugated bilirubin IXa and prevalence of the monoglucuronide over the digl ucuronide were found. Finally, DNA from liver donors' lymphocytes was avail able for one jaundiced and two nonjaundiced patients: tests for abnormaliti es in the promoter region of the gene for the enzyme bilirubin uridine diph ospho-glucuronosyltransferase were in agreement with a diagnosis of GS in t he former one. Conclusions: Gilbert syndrome may have an unusual early presentation in ped iatric OLT recipients.