Chronic Idiopathic Jaundice (Dubin Johnson Syndrome)

A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins. Affected persons may be asymptomatic or have vague constitutional or gastrointestinal symptoms. The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract.

Also Known As:

Networked: 141 relevant articles (7 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Jaundice
2.	Hereditary Hyperbilirubinemia (Rotor Syndrome)
3.	Liver Diseases (Liver Disease)
4.	Gilbert Disease
5.	Hepatocellular Carcinoma (Hepatoma)

Experts

1.	Hayashi, Hisao: 4 articles (10/2007 - 01/2003)
2.	Yano, Motoyoshi: 4 articles (10/2007 - 01/2003)
3.	Wakusawa, Shinya: 4 articles (10/2007 - 01/2003)
4.	Jirsa, Milan: 3 articles (02/2013 - 07/2005)
5.	Keppler, Dietrich: 3 articles (02/2007 - 07/2002)
6.	Machida, Ikuo: 3 articles (04/2005 - 01/2003)
7.	Jansen, P L: 3 articles (12/2000 - 01/2000)
8.	Uchiumi, Takeshi: 2 articles (05/2013 - 11/2002)
9.	Elleder, Milan: 2 articles (02/2013 - 07/2005)
10.	Mor-Cohen, Ronit: 2 articles (03/2007 - 02/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chronic Idiopathic Jaundice:

1.	Phenobarbital (Luminal)FDA Link 04/01/1996 - "Following phenobarbital administration, the ICG secondary rise in the 5 patients with Dubin-Johnson syndrome disappeared, and ICG was rapidly cleared from blood. " 04/01/1996 - "However, in patients with Dubin-Johnson syndrome, BSP clearance in serum did not show any change before and after phenobarbital administration. " 05/16/1976 - "[Phenobarbital in Dubin-Johnson syndrome]." 01/01/1976 - "The effect of phenobarbital on patients with Dubin-Johnson syndrome." 08/01/1974 - "Effect of phenobarbital on liver functions in patients with Dubin-Johnson syndrome."
2.	Ursodeoxycholic Acid (Urso)FDA LinkGeneric 04/27/1981 - "Abnormality of oral ursodeoxycholic acid tolerance test in the Dubin-Johnson syndrome." 07/01/1981 - "As the area under the curve reflects the hepatic clearance of intestinally absorbed ursodeoxycholic acid and therefore gives a quantitative estimate of hepatic ursodeoxycholic acid clearance, the hepatic clearance of ursodeoxycholic acid seems to be impaired in patients with Gilbert's syndrome and Dubin-Johnson syndrome. " 01/01/2002 - "We suggest ursodeoxycholic acid in treatment for Dubin-Johnson Syndrome with severe direct hyperbilirubinemia presenting in the neonatal age." 01/01/2002 - "Treatment of severe cholestasis in neonatal Dubin-Johnson syndrome with ursodeoxycholic acid." 07/01/1981 - "An oral tolerance test using a 25-mg dose of ursodeoxycholic acid was carried out in 46 patients with constitutional hyperbilirubinemias: 26 with Gilbert's syndrome, 9 with Dubin-Johnson syndrome, 7 with Rotor Syndrome, 3 with constitutional indocyanine green intolerance, and 1 with Crigler-Najjar syndrome type II; they were compared with 14 healthy control subjects. "
3.	Technetium Tc 99m LidofeninIBA 05/01/1983 - "99mTc-HIDA cholescintigraphy in children with Dubin-Johnson syndrome." 03/01/1982 - "It is concluded that 99mTc-HIDA cholescintigraphy may be helpful in the diagnosis of Dubin-Johnson syndrome and Rotor syndrome and in the differential diagnosis between these two conditions." 03/01/1982 - "99mTc-HIDA cholescintigraphy was performed in 6 patients with Dubin-Johnson syndrome and 1 patient with Rotor syndrome. "
4.	IronIBA 09/01/2004 - "In conclusion, deep jaundice in a patient with Dubin-Johnson syndrome complicated by hepatitis C virus-positive chronic liver disease and iron overload was partially improved by iron-reduction therapy." 01/01/1987 - "Patients with metal and pigment metabolic disorders include iron storage disease, either hemochromatosis or transfusional hemosiderosis, Menkes' kinky hair syndrome, and Dubin-Johnson syndrome. " 09/01/2004 - "Iron-reduction therapy by venesection, an alternative to interferon, was performed in a 55-year-old male patient with Dubin-Johnson syndrome complicated by hepatitis C virus-positive chronic liver disease and hepatic iron overload. "
5.	Rose BengalIBA 03/15/1973 - "[Excretion of rose bengal labeled with I 131 and plasma clearance of bromosulphalein in Dubin-Johnson syndrome]."
6.	Glycocholic AcidIBA 10/01/1980 - "Serum bile salt measurements and intravenous clearance of glycocholate were performed in a woman with Dubin-Johnson syndrome. "
7.	BilirubinIBA 06/01/2014 - "Impairment of bilirubin secretion leads to accumulation of conjugated bilirubin (Dubin-Johnson syndrome). " 01/01/2013 - "Dubin-Johnson syndrome (DJS) is a rare autosomal recessive inheritance disorder of bilirubin metabolism. " 02/01/2007 - "Acquired or hereditary deficiency of ABCC2, the latter known as Dubin-Johnson syndrome in humans, causes an increased concentration of bilirubin glucuronosides in blood because of their efflux from hepatocytes via the basolateral ABCC3, which compensates for the deficiency in ABCC2-mediated apical efflux. " 01/01/2003 - "Mutations in human MRP2 result in defects in excretion of conjugated bilirubin and other cholephiles known as the Dubin-Johnson syndrome. " 03/01/1998 - "These results demonstrate that SnMP can lower plasma bilirubin concentrations for extended periods in a new mutant rat model of Dubin-Johnson syndrome."
8.	Sulfobromophthalein (Sulfobromophthalein Sodium)IBA 10/31/1969 - "[Metabolism of sulfobromophthalein (BSF) in Dubin-Johnson syndrome (study of 4 cases)]." 02/01/1982 - "Impairment of bromosulfophthalein clearance by bile salt administration in normal volunteers and patients with Dubin-Johnson syndrome." 06/01/1972 - "Metabolism of the bromsulphalein in Dubin-Johnson syndrome. " 12/01/1970 - "Sulfobromophthalein tolerance test in patients with Dubin-Johnson syndrome and their relatives." 01/01/1963 - "[KINETICS OF BROMSULPHALEIN IN THE DUBIN-JOHNSON SYNDROME]."
9.	Adenosine Triphosphate (ATP)IBA 11/01/2002 - "Trafficking and functional defects by mutations of the ATP-binding domains in MRP2 in patients with Dubin-Johnson syndrome." 04/01/2002 - "A new mutation of the ATP-binding cassette, sub-family C, member 2 (ABCC2) gene in a Japanese patient with Dubin-Johnson syndrome." 10/01/2000 - "[Genomic structure of the canalicular multispecific organic anion-transporter gene (MRP2/cMOAT) and mutations in the ATP-binding-cassette region in Dubin-Johnson syndrome]." 03/01/1999 - "Genomic structure of the canalicular multispecific organic anion-transporter gene (MRP2/cMOAT) and mutations in the ATP-binding-cassette region in Dubin-Johnson syndrome." 06/01/1996 - "Hepatic excretion of non-bile acid organic anions is reported to be ATP-dependent and a defect of this transport has been reported in congenitally jaundiced rats, animal models of human Dubin-Johnson syndrome. "
10.	P-glycoprotein 2IBA 04/01/2005 - "Recent studies have indicated that dysfunction or loss of the multidrug resistance protein 2 (MRP2) is the molecular basis of Dubin-Johnson syndrome (DJS). " 05/01/2013 - "Mutation and functional analysis of ABCC2/multidrug resistance protein 2 in a Japanese patient with Dubin-Johnson syndrome." 02/01/2005 - "A novel ancestral splicing mutation in the multidrug resistance protein 2 gene causes Dubin-Johnson syndrome in Ashkenazi Jewish patients." 10/05/2001 - "Identification and functional analysis of two novel mutations in the multidrug resistance protein 2 gene in Israeli patients with Dubin-Johnson syndrome." 12/01/2000 - "Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin-Johnson syndrome."

Therapies and Procedures

1.	Segmental Mastectomy (Lumpectomy) 05/01/2004 - "Hepatocellular carcinoma in a case of Dubin-Johnson syndrome treated successfully with a central bilateral segmentectomy." 05/01/2004 - "A 72-year-old woman with hepatocellular carcinoma and HCV cirrhosis combined with Dubin-Johnson syndrome was successfully treated by a central bilateral segmentectomy. "
2.	Lasers (Laser) 05/01/1996 - "In the present study we show the selective absence of the canalicular isoform of MRP (cMRP) from the hepatocytes in a patient with Dubin-Johnson syndrome by double-label immunofluorescence and confocal laser scanning microscopy using antibodies directed against MRP and dipeptidyl-peptidase IV (DPPIV). "
3.	Aftercare (After-Treatment) 02/29/1976 - "[Study of bilirubin kinetics in a case of Dubin-Johnson syndrome before and after treatment with UDPG]."
4.	Hepatectomy 01/01/1998 - "Postoperative management following massive hepatectomy in a patient with Dubin-Johnson syndrome: report of a case." 02/01/1983 - "Patient with Dubin-Johnson syndrome tolerated major hepatectomy. " 02/01/2013 - "Hepatectomy in a hepatocellular carcinoma case with Dubin-Johnson syndrome and indocyanine green excretory defect."
5.	Transplants (Transplant) 02/01/2014 - "A liver biopsy was performed for hyperbilirubinemia 2 weeks after the orthotopic liver transplant that showed a Cryptococcus-like yeast. " 02/01/2012 - "Living donor liver transplantation using a graft from a donor with Dubin-Johnson syndrome."