eMedicine Specialties > Gastroenterology > Liver

Dubin-Johnson Syndrome: Treatment & Medication

Author: Samir L Habashi, MD, Assistant Professor of Medicine, Department of Medicine, Division of Gastroenterology, University of Florida at Jacksonville
Coauthor(s): Louis R Lambiase, MD, Associate Professor of Medicine, University of Florida College of Medicine; Chief, Division of Gastroenterology, Department of Internal Medicine, University of Florida Health Science Center/Jacksonville; Miriam K Anand, MD, Consulting Staff, Department of Allergy/Immunology, Allergy Associates and Lab, Ltd; Kenneth J Mishark, MD, Instructor, Department of Internal Medicine, Mayo Clinic Scottsdale, Mayo Medical School; Cuong Nguyen, MD, Instructor, Department of Internal Medicine, Section of Gastroenterology, Mayo Clinic Scottsdale
Contributor Information and Disclosures

Updated: May 19, 2009

Treatment

Medical Care

Dubin-Johnson syndrome (DJS) is a benign disorder and does not require any specific therapy. In the past, patients were treated with phenobarbital, which was primarily used to reduce serum bilirubin levels. This treatment is no longer recommended. Patients should be warned that pregnancy, oral contraceptive use, and intercurrent illness can exacerbate the icterus.

Drugs such as rifampicin and ursodeoxycholic acid (UDCA) should be used with caution in patients with Dubin-Johnson syndrome (DJS).  

Corpechot et al reported the effects of chronic administration of rifampicin and UDCA in a patient with Dubin-Johnson syndrome (DJS) having a novel 974C→G nonsense mutation of the MRP2/ABCC2 gene.9 The adverse effects of these drugs on serum bilirubin and bile acid concentrations may result from an increased expression of MRP3 at the basolateral membrane of hepatocytes as a direct consequence of drug induction. This observation suggests that these drugs should be used with caution in situations in which MRP2 expression may be decreased, as observed in the late stage of cholestasis.9

More on Dubin-Johnson Syndrome

Overview: Dubin-Johnson Syndrome
Differential Diagnoses & Workup: Dubin-Johnson Syndrome
Treatment & Medication: Dubin-Johnson Syndrome
Follow-up: Dubin-Johnson Syndrome
Multimedia: Dubin-Johnson Syndrome
References
Further Reading
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References

  1. Paulusma CC, Kool M, Bosma PJ, et al. A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndrome. Hepatology. Jun 1997;25(6):1539-42. [Medline]. [Full Text].

  2. Toh S, Wada M, Uchiumi T, et al. Genomic structure of the canalicular multispecific organic anion-transporter gene (MRP2/cMOAT) and mutations in the ATP-binding-cassette region in Dubin-Johnson syndrome. Am J Hum Genet. Mar 1999;64(3):739-46. [Medline]. [Full Text].

  3. Nisa AU, Ahmad Z. Dubin-Johnson syndrome. J Coll Physicians Surg Pak. Mar 2008;18(3):188-9. [Medline].

  4. Zlotogora J. Hereditary disorders among Iranian Jews. Am J Med Genet. Jul 31 1995;58(1):32-7. [Medline].

  5. Mor-Cohen R, Zivelin A, Fromovich-Amit Y, et al. Age estimates of ancestral mutations causing factor VII deficiency and Dubin-Johnson syndrome in Iranian and Moroccan Jews are consistent with ancient Jewish migrations. Blood Coagul Fibrinolysis. Mar 2007;18(2):139-44. [Medline].

  6. Bar-Meir S, Baron J, Seligson U, et al. 99mTc-HIDA cholescintigraphy in Dubin-Johnson and Rotor syndromes. Radiology. Mar 1982;142(3):743-6. [Medline]. [Full Text].

  7. Zimniak P. Dubin-Johnson and Rotor syndromes: molecular basis and pathogenesis. Semin Liver Dis. Aug 1993;13(3):248-60. [Medline].

  8. Sobaniec-Lotowska ME, Lebensztejn DM. Ultrastructure of Kupffer cells and hepatocytes in the Dubin-Johnson syndrome: a case report. World J Gastroenterol. Feb 14 2006;12(6):987-9. [Medline]. [Full Text].

  9. Corpechot C, Ping C, Wendum D, et al. Identification of a novel 974C-->G nonsense mutation of the MRP2/ABCC2 gene in a patient with Dubin-Johnson syndrome and analysis of the effects of rifampicin and ursodeoxycholic acid on serum bilirubin and bile acids. Am J Gastroenterol. Oct 2006;101(10):2427-32. [Medline].

  10. Bosia JD, D'Ascenzo MV, Borzi S, et al. [The Dubin-Johnson syndrome: case report and review of literature] [Spanish]. Acta Gastroenterol Latinoam. Sep 2008;38(3):194-8. [Medline].

  11. Dubin IN. Chronic idiopathic jaundice; a review of fifty cases. Am J Med. Feb 1958;24(2):268-92. [Medline].

  12. Fretzayas A, Kitsiou S, Papadopoulou A, Nicolaidou P. Clinical expression of co-inherited Dubin-Johnson and thalassaemic heterozygous states. Dig Liver Dis. Apr 2007;39(4):369-74. [Medline].

  13. Jung MK, Bae MH, Kim DJ, et al. [A case with Rotor syndrome in hyperbilirubinemic family] [Korean]. Korean J Gastroenterol. Apr 2007;49(4):251-5. [Medline]. [Full Text].

  14. Nowicki MJ, Poley JR. The hereditary hyperbilirubinaemias. Baillieres Clin Gastroenterol. Jun 1998;12(2):355-67. [Medline].

  15. Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome--a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. Oct 2006;49(4):500-4. [Medline].

  16. Shani M, Seligsohn U, Gilon E, Sheba C, Adam A. Dubin-Johnson syndrome in Israel. I. Clinical, laboratory, and genetic aspects of 101 cases. Q J Med. Oct 1970;39(156):549-67. [Medline].

  17. Sotelo-Avila C, Danis RK, Krafcik J, Malik M, Schwarz KB. Cholecystitis in a 17-year-old boy with recurrent jaundice since childhood. J Pediatr. Apr 1988;112(4):668-74. [Medline].

  18. The familial conjugated hyperbilirubinemias. Semin Liver Dis. Nov 1994;14(4):386-94. [Medline].

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Further Reading

Related eMedicine Topics

Clinical Trials
National Guideline Clearinghouse

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Keywords

Dubin-Johnson syndrome, DJS, hyperbilirubinemia, chronic idiopathic jaundice, neonatal jaundice, Gilbert syndrome, Crigler-Najjar syndrome, CNS, Rotor syndrome, Sprinz-Nelson syndrome

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Contributor Information and Disclosures

Author

Samir L Habashi, MD, Assistant Professor of Medicine, Department of Medicine, Division of Gastroenterology, University of Florida at Jacksonville
Samir L Habashi, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians-American Society of Internal Medicine, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Coauthor(s)

Louis R Lambiase, MD, Associate Professor of Medicine, University of Florida College of Medicine; Chief, Division of Gastroenterology, Department of Internal Medicine, University of Florida Health Science Center/Jacksonville
Louis R Lambiase, MD is a member of the following medical societies: American Gastroenterological Association, American Pancreatic Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Miriam K Anand, MD, Consulting Staff, Department of Allergy/Immunology, Allergy Associates and Lab, Ltd
Miriam K Anand, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians-American Society of Internal Medicine, and American Medical Association
Disclosure: Nothing to disclose.

Kenneth J Mishark, MD, Instructor, Department of Internal Medicine, Mayo Clinic Scottsdale, Mayo Medical School
Kenneth J Mishark, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Cuong Nguyen, MD, Instructor, Department of Internal Medicine, Section of Gastroenterology, Mayo Clinic Scottsdale
Disclosure: Nothing to disclose.

Medical Editor

Waqar A Qureshi, MD, Associate Professor of Medicine, Chief of Endoscopy, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine and Veterans Affairs Medical Center
Waqar A Qureshi, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

BS Anand, MD, Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine
BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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