Dubin-Johnson syndrome

Dubin-Johnson syndrome (DJS) is a benign, inherited liver disorder characterized clinically by chronic, predominantly conjugated, hyperbilirubinemia and histopathologically by black-brown pigment deposition in parenchymal liver cells.

Hyperbilirubinemia

An increased amount of bilirubin in the blood.


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PMID (PMCID)
21948575
 MALE Middle Aged
Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report.
Korkmaz U, Duman AE, Ogutmen Koc D, Gurbuz Y, Dndar G, Ensaroglu F, Sener SY, Senturk O, Hulagu S.
Turk J Gastroenterol. 2011;22(4):422-5.
In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis.
21948575
 MALE Middle Aged
Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report.
Korkmaz U, Duman AE, Ogutmen Koc D, Gurbuz Y, Dndar G, Ensaroglu F, Sener SY, Senturk O, Hulagu S.
Turk J Gastroenterol. 2011;22(4):422-5.
We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia.
18979899
 MALE Adult
[The Dubin-Johnson syndrome: case report and review of literature].
Bosia JD, D'Ascenzo MV, Borzi S, Cozzi S, Defelitto JR, Curciarello JO.
Acta Gastroenterol Latinoam. 2008;38(3):194-8.
The Dubin-Johnson syndrome is a hereditary deficiency in the excretion ofconjugated bilirrubin by hepatocytes characterized by chronic hyperbilirubinemia, alteration in coproporphyrin metabolism, and intracellular deposition of a dark melanin-like pigment giving the liver a typical black cast.
15565411
 MALE Middle Aged
Improvement of serum bilirubin levels after venesection in a patient with Dubin-Johnson syndrome and HCV-positive chronic liver disease.
Machida I, Wakusawa S, Hayashi H, Suzuki S, Yano M.
J Gastroenterol. 2004;39(9):896-9.
Direct-type hyperbilirubinemia in Dubin-Johnson syndrome is due to the genetic dysfunction of multidrug resistance protein 2.
12012642
 MALE Infant, Newborn
Treatment of severe cholestasis in neonatal Dubin-Johnson syndrome with ursodeoxycholic acid.
Regev RH, Stolar O, Raz A, Dolfin T.
J Perinat Med. 2002;30(2):185-7.
We report a case of Dubin-Johnson Syndrome in a neonate presenting with severe direct hyperbilirubinemia, which failed to respond to phenobarbital treatment.
12012642
 MALE Infant, Newborn
Treatment of severe cholestasis in neonatal Dubin-Johnson syndrome with ursodeoxycholic acid.
Regev RH, Stolar O, Raz A, Dolfin T.
J Perinat Med. 2002;30(2):185-7.
We suggest ursodeoxycholic acid in treatment for Dubin-Johnson Syndrome with severe direct hyperbilirubinemia presenting in the neonatal age.