Gilbert Disease

A benign familial disorder, transmitted as an autosomal dominant trait. It is characterized by low-grade chronic hyperbilirubinemia with considerable daily fluctuations of the bilirubin level.

Also Known As:

Constitutional Liver Dysfunction; Familial Nonhemolytic Jaundice; Gilbert Syndrome; Gilbert's Disease; Gilbert's Syndrome; Gilbert-Lereboullet Syndrome; Hyperbilirubinemia 1; Hyperbilirubinemia I; Hyperbilirubinemia, Arias Type; Meulengracht Syndrome; Unconjugated Benign Bilirubinemia; Arias Type Hyperbilirubinemia; Arias Type Hyperbilirubinemias; Disease, Gilbert; Disease, Gilbert's; Gilberts Disease; Gilberts Syndrome; Hyperbilirubinemia 1s; Hyperbilirubinemias, Arias Type; Syndrome, Gilbert; Syndrome, Gilbert's

Networked: 635 relevant articles (22 outcomes, 62 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hyperbilirubinemia
2.	Crigler-Najjar Syndrome
3.	Cardiovascular Diseases (Cardiovascular Disease)
4.	Chronic Idiopathic Jaundice (Dubin Johnson Syndrome)
5.	Infections

Experts

1.	Wagner, Karl-Heinz: 14 articles (12/2021 - 05/2012)
2.	Bulmer, Andrew C: 12 articles (12/2021 - 08/2008)
3.	Strassburg, Christian P: 11 articles (01/2020 - 05/2005)
4.	Doberer, Daniel: 8 articles (12/2021 - 09/2013)
5.	Marculescu, Rodrig: 8 articles (12/2021 - 09/2013)
6.	Vítek, Libor: 7 articles (06/2022 - 02/2002)
7.	Mölzer, Christine: 7 articles (12/2021 - 09/2013)
8.	Maruo, Yoshihiro: 7 articles (02/2016 - 01/2002)
9.	Wallner, Marlies: 6 articles (01/2018 - 09/2013)
10.	Sato, Hiroshi: 6 articles (02/2016 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Gilbert Disease:

1.	BilirubinIBA 07/01/2023 - "However, for milder elevations of unconjugated bilirubin (suspicious for Gilbert disease) in the outpatient setting, a US demonstrating lack of biliary dilatation was often a definitive study for exclusion of pathology." 06/27/2022 - "The purpose of the current study was to evaluate serum bilirubin concentrations and the prevalence of Gilbert syndrome (GS) in elite athletes. " 01/01/2021 - "This study investigated, whether gut microbiota composition is altered in Gilbert's Syndrome (GS), a mild form of chronically elevated serum unconjugated bilirubin (UCB) compared to matched controls. " 11/01/2014 - "To investigate the effect of age on micronuclei (MN) frequency and evaluate the particular age group, our data were merged with data from a recent study by Wallner et al. (Effects of unconjugated bilirubin on chromosomal damage in individuals with Gilbert's syndrome measured with the micronucleus cytome assay. " 01/01/2013 - "We aimed to investigate serum bilirubin levels and arterial stiffness parameters in patients with Gilbert's syndrome in this study. "
2.	Niacin (Nicotinic Acid)FDA LinkGeneric 04/01/1991 - "It has been suggested that the nicotinic acid provocation test is useful in the diagnosis of Gilbert's syndrome. " 03/01/1990 - "The hyperbilirubinaemic effect of nicotinic acid was confirmed to be more pronounced in Gilbert's syndrome patients than in controls. " 03/01/1990 - "Dissociation between vascular and metabolic effects of nicotinic acid in Gilbert's syndrome." 08/01/1987 - "Hypersideremic and hyperbilirubinemic effect of nicotinic acid in patients with Gilbert's syndrome." 10/01/1981 - "[Summary of experiences with the nicotinic acid and low-calorie tests in Gilbert's syndrome]."
3.	Phenobarbital (Luminal)FDA Link 03/01/2016 - "The T-3279G mutation in the phenobarbital responsive enhancer module (PBREM), the TA-insertion in the TATA box, creating the A(TA)7TAA motif instead of A(TA)6TAA and the G211A mutation in coding exon 1, particularly in Asian populations, of the human UGT1A1 gene are the three common genotypes found in patients with Gilbert's syndrome. " 04/01/2005 - "Recently, it was reported that the -3263T > G mutation in the phenobarbital response enhancer module in UGT1A1 was associated with the majority of cases of Gilbert syndrome. " 10/01/1989 - "Phenobarbital induction appears to be the most suitable and reliable test for clinical non-invasive diagnosis of Gilbert's syndrome." 06/01/1988 - "[The use of optimal doses of phenobarbital in the treatment of Gilbert's syndrome]." 01/01/1981 - "The diagnostic role of the reduced caloric intake test and phenobarbitone treatment in Gilbert's syndrome was evaluated. "
4.	rifamycin SVIBA 06/01/1985 - "Impaired plasma clearance of nicotinic acid and rifamycin-SV in Gilbert's syndrome: evidence of a functional heterogeneity." 04/01/1984 - "Plasma clearance of nicotinic acid and rifamycin-SV, and their interaction in Gilbert's syndrome: application of a compartmental model." 08/01/1990 - "Competition studies with combined administration of sulfobromophthalein (0.59 mumol/kg) and different doses of rifamycin SV (0.59, 1.47 and 2.95 mumol/kg) showed a significant (p less than 0.001) reduction in plasma disappearance rate in Gilbert's syndrome patients but not in controls. " 08/01/1990 - "The rifamycin SV dose at which a 50% inhibition in plasma disappearance rate was observed was 0.8 mumol/kg. The apparent affinity (Km) of the hepatic transport was higher in Gilbert's syndrome patients than in control subjects (3.61 +/- 0.37 mumol sulfobromophthalein/kg vs. 2.76 +/- 0.29 mumol sulfobromophthalein/kg, mean +/- S.D.; p less than 0.01), whereas no difference was found in Vmax (0.95 +/- 0.11 mumol sulfobromophthalein/kg vs. 0.93 +/- 0.10 mumol sulfobromophthalein/kg/min, mean +/- S.D.; N.S.).(ABSTRACT TRUNCATED AT 250 WORDS)" 06/01/1985 - "The implication of bilitranslocase function in the impaired rifamycin SV metabolism in Gilbert's syndrome."
5.	bilirubin glucuronoside glucuronosyltransferaseIBA 06/08/2010 - "Gilbert syndrome is caused by defects in bilirubin UDP-glucuronosyltransferase (UGT1A1). " 10/01/2007 - "Gilbert's syndrome (GS) is caused by a reduction in the activity of hepatic bilirubin UDP-glucuronosyltransferase (UGT). " 04/14/2006 - "Gilbert's syndrome: High frequency of the (TA)7 TAA allele in India and its interaction with a novel CAT insertion in promoter of the gene for bilirubin UDP-glucuronosyltransferase 1 gene." 05/01/2002 - "A patient suffering from Gilbert's syndrome may have hepatic activity of bilirubin-UDP-glucuronosyltransferase decreasing to levels around 30% of the normal. " 04/01/2001 - "Mutations in the promoter as well as in the coding region of the bilirubin UDP-glucuronosyltransferase gene (UGT1A1) have been found to be associated with Gilbert's syndrome. "
6.	Isotretinoin (Accutane)FDA LinkGeneric 01/01/1997 - "However, considering the prevalence of Gilbert's syndrome and its usual first expression during postpubertal period, it seems to us interesting for therapeutic practice to know that isotretinoin is not less safe in these patients." 01/01/1997 - "Safety of isotretinoin in Gilbert's syndrome was first observed in 1984, but its beneficial effects have only recently been described by Wang et al., and we report here a similar case. " 01/01/1997 - "[Gilbert disease and isotretinoin]." 01/01/1997 - "We report here a new case of good tolerance and even improvement of a Gilbert's syndrome during isotretinoin therapy. " 03/20/2014 - "Hepatotoxicity of isotretinoin in patients with acne and Gilbert's syndrome: a comparative study."
7.	Sulfobromophthalein (Sulfobromophthalein Sodium)IBA 08/01/1990 - "Significant reduction was also found after administration to four Gilbert's syndrome patients and four control subjects of 0.29 and 2.95 mumol sulfobromophthalein (0.060 +/- 0.005 mumol/kg/min vs. 0.077 +/- 0.07 mumol/kg/min and 0.480 +/- 0.012 mumol/kg/min vs. 0.591 +/- 0.015 mumol/kg/min, respectively; p less than 0.01). " 01/01/1997 - "Plasma sulfobromophthalein disappearance in Gilbert's syndrome." 08/01/1990 - "Plasma disappearance rate was significantly reduced in Gilbert's syndrome patients after administration of 0.59 mumol sulfobromophthalein/kg (0.119 +/- 0.016 vs. 0.146 +/- 0.018 mumol/kg/min; mean +/- S.D.; p less than 0.001), whereas no difference was found with the higher dose (0.754 +/- 0.040 vs. 0.767 +/- 0.072 mumol/kg/min). " 09/01/1972 - "Defective bromosulfophthalein clearance in patients with constitutional hepatic dysfunction (Gilbert's syndrome)." 01/01/1975 - "[Bromsulphalein clearance and hyperbilirubinemia in Gilbert's syndrome]."
8.	oxidized low density lipoproteinIBA 03/20/2014 - "LDL levels were increased in the two study groups, but this increase was less substantial in patients with Gilbert's syndrome. " 03/01/2011 - "Decreased small dense LDL levels in Gilbert's syndrome." 01/01/2011 - "We found out that the protective antiatherogenic effect of hyperbilirubinemia is potentiated by low occurence of strongly atherogenic small dense LDL and persons with byperbilirubionemia (in our case represented Gilbert's syndrome), could be protected against the development of atheroscleosis." 05/15/2012 - "Reduced circulating oxidized LDL is associated with hypocholesterolemia and enhanced thiol status in Gilbert syndrome." 03/01/2011 - "To investigate the role of small dense low density lipoprotein cholesterol (sd-LDL-C) in the mechanism of decreased incidence of cardiovascular disease in Gilbert's syndrome (GS). "
9.	Lipoproteins (Lipoprotein)IBA 01/01/2011 - "Probands with Gilbert's syndrome had significantly lower presence of atherogenic lipoprotein spectrum than probands in control group (5% vs. 18%). " 05/15/2012 - "The aim of this study was to assess whether persons expressing Gilbert syndrome (GS; i.e., unconjugated hyperbilirubinemia) are protected from thiol oxidation and to determine if this, in addition to their improved lipoprotein profile, could explain reduced oxidized low-density lipoprotein (oxLDL) status in them. " 01/01/2011 - "The aim of this study was to analyze the serum bilirubin level, lipid and lipoprotein parameters with the emphasis on the presence of atherogenic small dense LDL in patients with Gilbert's syndrome and to compare these results with probands in the control group. "
10.	AnionsIBA 03/01/1976 - "These studies confirm previous work indicating that patients with Gilbert's syndrome constitute a heterogeneous population with regard to defects in hepatic organic anion transport, some of the defects not being attributable to glucuronyl transferese deficiency. " 06/01/1985 - "Patients with Gilbert's syndrome (GS) have impaired clearance by the liver of some organic anions. " 07/01/1981 - "These patients manifest a noval disorder of organic anion transport which, like Gilbert's syndrome, is also characterized by chronic milk unconjugated hyperbilirubinemia." 03/01/2001 - "Hepatic uptake of organic anions affects the plasma bilirubin level in subjects with Gilbert's syndrome mutations in UGT1A1." 08/01/1990 - "Abnormal hepatic uptake of low doses of sulfobromophthalein in Gilbert's syndrome: the role of reduced affinity of the plasma membrane carrier of organic anions."

Therapies and Procedures

1.	Therapeutics 07/01/2015 - "Exclusion criteria were switch in antiretroviral therapy during the previous 4 months, previous virological failure, pregnancy or breastfeeding, Gilbert's syndrome, use of contraindicated drugs, grade 4 laboratory abnormalities, and previous intolerance to any of the study drugs. " 06/01/2006 - "Gilbert syndrome and the development of antiretroviral therapy-associated hyperbilirubinemia: genetic screening is unnecessary." 10/15/2005 - "Gilbert syndrome and the development of antiretroviral therapy-associated hyperbilirubinemia." 07/01/2009 - "A genetic disposition for Gilbert's syndrome explained the adverse events and permitted a continuation of therapy leading to a sustained clearance of chronic hepatitis C infection. " 01/01/2014 - "[Effect of anti-helicobacter therapy on the hepatic glucuronyl transferase system of adolescents with Gilbert's syndrome]."
2.	Splenectomy 07/01/1981 - "In the remaining seven (group B), low preoperative values for CBR, suggestive of Gilbert's syndrome, uniformly improved after splenectomy, becoming normal in five patients. " 08/01/2004 - "There was a statistically significant association between splenectomy and both cholelithiasis (P = 0.022) and Gilbert syndrome (P = 0.002). " 08/01/2004 - "Increased prevalence of cholelithiasis in Gaucher disease: association with splenectomy but not with gilbert syndrome."
3.	Liver Transplantation 09/01/1994 - "This study was conducted to determine the frequency of Gilbert's syndrome in liver transplant recipients. " 01/01/2023 - "Gilbert's syndrome in a liver transplant recipient with AIH-PBC overlap syndrome." 01/01/2020 - "We evaluate whether it is safe to accept donors with Gilbert's syndrome (GS) for a living donor liver transplantation (LDLT) or not. " 01/01/2020 - "Adult living donor liver transplantation from donors with Gilbert's syndrome: Is it safe?" 12/01/2015 - "Donor Safety and Recipient Liver Function After Right-Lobe Liver Transplantation From Living Donors With Gilbert Syndrome."
4.	Oral Administration 01/01/2002 - "This study showed that acute and chronic oral administration of ZnSO4 decreased serum UCB levels significantly in subjects with Gilbert s syndrome. " 05/31/1978 - "The pahrmacokinetics of paracetamol after intravenous and oral administration has been studied in 6 patients with Gilbert's syndrome, and 6 healthy controls. " 07/01/1981 - "The integrated area under the curve of increments of serum UDCA above the base line between 0 and 2 h after oral administration was significantly increased in patients with Gilbert's syndrome and Dubin-Johnson syndrome, whereas it was normal in patients with Rotor syndrome and constitutional indocyanine green intolerance. "
5.	Hepatectomy 02/01/2012 - "The purpose of this study was to assess the results of right-lobe living-donor hepatectomy of liver donors with Gilbert syndrome. "