Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome)
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.
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Meulengracht-Gilbert Syndrome (MGS) is a benign genetic disorder present in approximately 10% of young men in Bavaria, and it affects bilirubin processing leading to elevated levels of unconjugated bilirubin in the blood.
“Meulengracht-Gilbert Syndrome (MGS), a benign genetic disorder which is present in approximately 10% of young men in Bavaria and affects bilirubin processing leading to elevated levels of unconjugated bilirubin in the blood [14], [15], [16].”
From Proof-of-concept of a low-dose unmodified mRNA-based rabies vaccine formulated with lipid nanoparticles in human volunteers: A phase 1 trial (2021) · cites this paper as [15]
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- 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 ›
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