Cited by 184Neonatal Health and BiochemistryPediatric Hepatobiliary Diseases and TreatmentsMetabolism and Genetic Disorders

Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome)

Strassburg, Christian P.·2010·Best Practice & Research Clinical Gastroenterology

Abstract

Hyperbilirubinemia is an important clinical sign that often indicates severe hepatobiliary disease of different etiologies. Inherited non-haemolytichyperbilirubinemic conditions include Dubin-Johnson, Rotor, and Gilbert-Meulengracht syndromes, which are important differential diagnoses indicating benign disease that require no immediate treatment. Dubin-Johnson and Rotor syndromes are rare, exhibit mixed direct and indirect hyperbilirubinemia as well as typical profiles or urinary coproporphyrin excretion. Gilbert-Meulengracht disease leads to unconjugated hyperbilirubinemia because of impaired glucuronidation activity, and is part of a spectrum of genetic variants also encompassing fatal Crigler-Najjar syndrome. Gilbert-Meulengracht syndrome can be diagnosed by clinical presentation, biochemistry and genotyping, and carries significance regarding the disposition towards drug-associated toxicity. In addition, the precise diagnosis of these inherited hyperbilirubinemic syndromes avoids unnecessary invasive procedures for suspected more severe hepatobiliary disease.

Integrity signals

Registry

Crossref · 10.1016/j.bpg.2010.07.007

Cites

98 of 98 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Strassburg, C. P.
Affiliations
Medizinische Hochschule Hannover (DE)
Corresponding author
Strassburg, Christian P.
Venue
Best Practice & Research Clinical Gastroenterology
Publisher
Elsevier BV
Published
2010-10
Volume
24
Issue
5
Pages
555-571
ISSN
1521-6918 (ISSN-L)
OpenAlex
W2030075454
Cited by
184
References
98
Citations by year
2026: 13 · 2025: 19 · 2024: 13 · 2023: 18 · 2022: 8 · 2021: 12 · 2020: 17 · 2019: 13 · 2018: 7 · 2017: 7 · 2016: 9 · 2015: 7 · 2014: 12 · 2013: 7 · 2012: 14
FWCI
1.32
Type
journal article
Language
en
Open access
closed
Locations
  • Best Practice & Research Clinical Gastroenterology (publishedVersion)
  • PubMed (publishedVersion)
Funders
Deutsche Forschungsgemeinschaft
Topics
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Neonatal Health and Biochemistry
  • Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
hyperbilirubinemia, Gilbert syndrome, Crigler-Najjar syndrome, Dubin-Johnson syndrome, Rotor syndrome, unconjugated hyperbilirubinemia, conjugated hyperbilirubinemia, glucuronidation, differential diagnosis, drug toxicity, genotyping
MeSH terms
Irinotecan, Animals, Antineoplastic Agents, Phytogenic, Bilirubin, Camptothecin, Crigler-Najjar Syndrome, Diagnosis, Differential, Gilbert Disease, Humans, Hyperbilirubinemia, Hereditary, Jaundice, Chronic Idiopathic, Neoplasms, Glucuronosyltransferase, Genetic Variation, Genetic Predisposition to Disease

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review