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

Pietro Vajro, Anna De Vincenzo, Stefania Lucariello, Fiorella Migliaro, Etienne Sokal, Olivier Bernard, Teresa Vilei, Maurizio Muraca

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Gilbert syndrome as a rule becomes manifest in adolescence or in early adulthood; it may be transferred by the donor to orthotopic liver transplant (OLT) recipients. Methods: We examined the frequency of Gilbert syndrome in 46 OLT pediatric recipients who had a follow-up of 1 year or more; Diagnostic criteria included unexplained chronic or recurrent unconjugated hyperbilirubinemia; its increase after reduced caloric intake plus prolonged fasting, without changes 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 otherwise healthy OLT recipients showed hyperbilirubinemia and normal conjugated fractions. Liver donors had been four men. Hyperbilirubinemia persisted with a fluctuating pattern for the whole follow-up after OLT in all. Total bilirubin level in blood samples obtained after reduced caloric intake and prolonged fasting became notably higher than basal values, whereas the proportion of conjugated bilirubin remained stable. High relative amounts of unconjugated bilirubin IXa and prevalence of the monoglucuronide over the diglucuronide were found. Finally, DNA from liver donors' lymphocytes was available for one jaundiced and two nonjaundiced patients: tests for abnormalities in the promoter region of the gene for the enzyme bilirubin uridine diphospho-glucuronosyltransferase were in agreement with a diagnosis of GS in the former one. Conclusions: Gilbert syndrome may have an unusual early presentation in pediatric OLT recipients. (C) 2000 Lippincott Williams and Wilkins, Inc.

Original languageEnglish
Pages (from-to)238-243
Number of pages6
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume31
Issue number3
DOIs
Publication statusPublished - 2000

Fingerprint

Gilbert Disease
liver transplant
bilirubin
Bilirubin
Liver Transplantation
Pediatrics
hyperbilirubinemia
Liver
Hyperbilirubinemia
Tissue Donors
Energy Intake
fasting
Fasting
energy intake
Glucuronosyltransferase
liver
uridine
Uridine
Jaundice
blood serum

Keywords

  • Children
  • Gilbert syndrome
  • Liver transplantation

ASJC Scopus subject areas

  • Gastroenterology
  • Histology
  • Medicine (miscellaneous)
  • Food Science
  • Pediatrics, Perinatology, and Child Health

Cite this

Unusual early presentation of Gilbert syndrome in pediatric recipients of liver transplantation. / Vajro, Pietro; De Vincenzo, Anna; Lucariello, Stefania; Migliaro, Fiorella; Sokal, Etienne; Bernard, Olivier; Vilei, Teresa; Muraca, Maurizio.

In: Journal of Pediatric Gastroenterology and Nutrition, Vol. 31, No. 3, 2000, p. 238-243.

Research output: Contribution to journalArticle

Vajro, P, De Vincenzo, A, Lucariello, S, Migliaro, F, Sokal, E, Bernard, O, Vilei, T & Muraca, M 2000, 'Unusual early presentation of Gilbert syndrome in pediatric recipients of liver transplantation', Journal of Pediatric Gastroenterology and Nutrition, vol. 31, no. 3, pp. 238-243. https://doi.org/10.1097/00005176-200009000-00007
Vajro, Pietro ; De Vincenzo, Anna ; Lucariello, Stefania ; Migliaro, Fiorella ; Sokal, Etienne ; Bernard, Olivier ; Vilei, Teresa ; Muraca, Maurizio. / Unusual early presentation of Gilbert syndrome in pediatric recipients of liver transplantation. In: Journal of Pediatric Gastroenterology and Nutrition. 2000 ; Vol. 31, No. 3. pp. 238-243.
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AU - Vajro, Pietro

AU - De Vincenzo, Anna

AU - Lucariello, Stefania

AU - Migliaro, Fiorella

AU - Sokal, Etienne

AU - Bernard, Olivier

AU - Vilei, Teresa

AU - Muraca, Maurizio

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N2 - Background: Gilbert syndrome as a rule becomes manifest in adolescence or in early adulthood; it may be transferred by the donor to orthotopic liver transplant (OLT) recipients. Methods: We examined the frequency of Gilbert syndrome in 46 OLT pediatric recipients who had a follow-up of 1 year or more; Diagnostic criteria included unexplained chronic or recurrent unconjugated hyperbilirubinemia; its increase after reduced caloric intake plus prolonged fasting, without changes 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 otherwise healthy OLT recipients showed hyperbilirubinemia and normal conjugated fractions. Liver donors had been four men. Hyperbilirubinemia persisted with a fluctuating pattern for the whole follow-up after OLT in all. Total bilirubin level in blood samples obtained after reduced caloric intake and prolonged fasting became notably higher than basal values, whereas the proportion of conjugated bilirubin remained stable. High relative amounts of unconjugated bilirubin IXa and prevalence of the monoglucuronide over the diglucuronide were found. Finally, DNA from liver donors' lymphocytes was available for one jaundiced and two nonjaundiced patients: tests for abnormalities in the promoter region of the gene for the enzyme bilirubin uridine diphospho-glucuronosyltransferase were in agreement with a diagnosis of GS in the former one. Conclusions: Gilbert syndrome may have an unusual early presentation in pediatric OLT recipients. (C) 2000 Lippincott Williams and Wilkins, Inc.

AB - Background: Gilbert syndrome as a rule becomes manifest in adolescence or in early adulthood; it may be transferred by the donor to orthotopic liver transplant (OLT) recipients. Methods: We examined the frequency of Gilbert syndrome in 46 OLT pediatric recipients who had a follow-up of 1 year or more; Diagnostic criteria included unexplained chronic or recurrent unconjugated hyperbilirubinemia; its increase after reduced caloric intake plus prolonged fasting, without changes 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 otherwise healthy OLT recipients showed hyperbilirubinemia and normal conjugated fractions. Liver donors had been four men. Hyperbilirubinemia persisted with a fluctuating pattern for the whole follow-up after OLT in all. Total bilirubin level in blood samples obtained after reduced caloric intake and prolonged fasting became notably higher than basal values, whereas the proportion of conjugated bilirubin remained stable. High relative amounts of unconjugated bilirubin IXa and prevalence of the monoglucuronide over the diglucuronide were found. Finally, DNA from liver donors' lymphocytes was available for one jaundiced and two nonjaundiced patients: tests for abnormalities in the promoter region of the gene for the enzyme bilirubin uridine diphospho-glucuronosyltransferase were in agreement with a diagnosis of GS in the former one. Conclusions: Gilbert syndrome may have an unusual early presentation in pediatric OLT recipients. (C) 2000 Lippincott Williams and Wilkins, Inc.

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