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Gilbert Disease

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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:

Gilbert Syndrome; Gilbert's Disease; Gilbert's Syndrome; Hyperbilirubinemia 1; Hyperbilirubinemia, Arias Type; 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: 481 relevant articles (19 outcomes, 49 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Strassburg, Christian P: 8 articles (05/2009 - 05/2005)
2.	Bulmer, Andrew C: 7 articles (03/2015 - 08/2008)
3.	Maruo, Yoshihiro: 6 articles (03/2011 - 01/2002)
4.	Kaplan, Michael: 6 articles (04/2009 - 04/2002)
5.	Wagner, Karl-Heinz: 5 articles (11/2014 - 05/2012)
6.	Sato, Hiroshi: 5 articles (01/2010 - 01/2002)
7.	Lankisch, Tim O: 5 articles (05/2009 - 05/2005)
8.	Manns, Michael P: 5 articles (05/2009 - 05/2005)
9.	Kaplan, M: 5 articles (12/2001 - 01/2000)
10.	Jirsa, Milan: 4 articles (10/2015 - 02/2002)

Drugs and Biologics

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

1.	BilirubinIBA 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. " 01/01/1993 - "The BoSM provides the investigator with an excellent animal model for human Gilbert's syndrome type I in which to study rate-limiting mechanisms in the transport of bilirubin from plasma to bile." 02/01/1992 - "The Bolivian squirrel monkey provides an excellent animal model for human Gilbert's syndrome type I in which to study rate-limiting mechanisms in the movement of bilirubin from plasma to bile." 11/10/1985 - "The results of a study conducted both on subjects affected by constitutional hyperbilirubinaemia (Gilbert's syndrome) and healthy subjects using the charge test with crystalline bilirubin are presented. "
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 01/01/2015 - "Sequencing of the Gilbert syndrome-associated gene, UGT 1A 1, revealed mutations in the upstream promoter phenobarbital-responsive element module (PBREM) (-3279 mutation, 23 cases), in the promoter TATA box (a TA insertion mutation, 21 cases), and in the coding region of exon 1 (a GGA-AGA Gly71Arg mutation, 18 cases); there was no statistical difference found for any of the three mutations among this patient population (x2 =1.640, P more than 0.05). " 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 - "1. The plasma disappearance rate and the increment in plasma unconjugated bilirubin after intravenous administration of 5.9 mumol of rifamycin SV (RSV)/kg body wt. were investigated in 51 subjects with Gilbert's syndrome and 35 control subjects of both sexes. "
5.	bilirubin glucuronoside glucuronosyltransferaseIBA 01/01/2010 - "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.	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)." 08/01/1990 - "The plasma disappearance rate of sulfobromophthalein (VBSP; mumol/kg/min) was measured in 15 Gilbert's syndrome patients and 12 control subjects after intravenous injection of two different doses (0.59 and 5.90 mumol/kg) of the dye. "
7.	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. " 07/31/2012 - "In addition, serum concentration of bilirubin correlated with malondialdehyde-modified low-density lipoprotein (r=-0.20, P=0.04) and 8-OHdG (r=-0.21, P=0.02) in patients with Gilbert syndrome but not in control subjects. " 07/31/2012 - "Flow-mediated vasodilation correlated with serum concentration of bilirubin (r=0.44, P<0.001), malondialdehyde-modified low-density lipoprotein (r=-0.25, P=0.01), and urinary excretion of 8-OHdG (r=-0.27, P=0.004) in patients with Gilbert syndrome but not in control subjects. "
8.	oxidized low density lipoproteinIBA 05/15/2012 - "Reduced circulating oxidized LDL is associated with hypocholesterolemia and enhanced thiol status in Gilbert syndrome." 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. "
9.	IronIBA 01/01/1973 - "Iron metabolism and storage in Gilbert's disease, and in Rotor's and Dubin-Johnson's syndromes." 06/01/2015 - "One patient had hereditary (-HFE) hemochromatosis complicated with Gilbert's syndrome, for which the pattern of iron deposition was similar to that of the four patients with Gilbert's syndrome. " 09/01/2013 - "Gilbert's syndrome subjects had significantly greater levels of unconjugated bilirubin (P < 0.05), carboxy haemoglobin (P < 0.05), iron (P < 0.05), IL-1β (P < 0.05), a significantly lower body mass index (P < 0.05) and IL-6 concentrations (P < 0.05) vs. controls. " 02/01/1986 - "The serum increments in unconjugated bilirubin and total iron were determined after intravenous administration of 5.90 mumol/kg body weight of nicotinic acid (NA) in 26 patients with Gilbert's syndrome (GS), seven patients with hemolytic anemia, and 13 healthy volunteers. " 11/01/1988 - "Nicotinic acid (NA) administration in Gilbert's syndrome (GS) patients promotes an increment of bilirubin and of total iron serum levels, dependent on a defective hepatic bilitranslocase function and on a haemolytic effect of NA. "
10.	AnionsIBA 06/01/1985 - "Patients with Gilbert's syndrome (GS) have impaired clearance by the liver of some organic anions. " 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." 04/01/1984 - "The bicompartmental kinetics of nicotinic acid (NA) and rifamycin-SV (R-SV)--2 organic anions that probably share a common hepatic uptake mechanism--were studied in 7 cases of Gilbert's syndrome (GS) and in 7 healthy controls matched for sex and age. " 10/01/1988 - "S-adenosyl-L-methionine might therefore prove effective in improving the efficiency of the transport of organic anions such as nicotinic acid (NA) and bilirubin which is impaired in Gilbert's syndrome (GS). "

Therapies and Procedures

1.	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."
2.	Transplants (Transplant) 12/01/2015 - "This study intended to assess donor safety and recipient liver function after LDLT with the use of right liver grafts from living donors with Gilbert syndrome. " 03/01/2012 - "The analysis of the promoter region of the UGT1A1-gene of the liver grafts showed three homozygous A(TA)(7) TAA alleles (homozygous Gilbert's syndrome) and one heterozygous A(TA)(7) TAA allele (heterozygous Gilbert's syndrome). " 02/01/2012 - "Living-donor liver transplant from Gilbert syndrome donors can be safely performed." 02/01/2012 - "Six of 225 right-lobe living-donor liver transplants were performed using donors with Gilbert syndrome. " 02/01/2012 - "Right-lobe liver transplant from donors with Gilbert syndrome."
3.	Transplantation (Transplant Recipients) 09/01/1994 - "This study was conducted to determine the frequency of Gilbert's syndrome in liver transplant recipients. " 09/01/1994 - "This study was performed to determine the incidence of unconjugated hyperbilirubinemia and particularly to determine the incidence of Gilbert's disease in liver transplant recipients. " 03/01/2012 - "This study shows that pediatric liver transplant recipients with unconjugated hyperbilirubinemia are very likely to have received a liver graft from a donor with Gilbert's syndrome." 05/15/2000 - "Our aim was to determine whether isolated hyperbilirubinemia in liver transplant recipients was due to Gilbert's syndrome acquired through the liver allograft. " 05/15/2000 - "Donor liver uridine diphosphate (UDP)-glucuronosyltransferase-1A1 deficiency causing Gilbert's syndrome in liver transplant recipients."
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. "