Korean J Gastroenterol.  2012 Apr;59(4):313-316. 10.4166/kjg.2012.59.4.313.

A Case of Sustained Cholestasis Caused by Acute A Viral Hepatitis in Dubin-Johnson Syndrome

• Ra SH
• Sung SY
• Jung HY
• Cha JH
• Baik SK
• Cho MY
• Kim MY

• KMID: 1364836
• DOI: http://doi.org/10.4166/kjg.2012.59.4.313

Abstract

Dubin-Johnson syndrome is a rare clinical entity. It shows intermittent symptoms such as chronic or intermittent jaundice, abdominal pain, weakness, nausea, vomiting, anorexia and diarrhea. Symptoms are precipitated or aggravated by pregnancy, alcoholism, surgical procedures and intercurrent disease. Chronic idiopathic jaundice is typical of Dubin-Johnson syndrome and its prognosis is good. We describe a case of prolonged cholestasis for more than 10 months caused by acute A viral hepatitis in a patient with Dubin-Johnson syndrome. It is a first report of cholestasis complicated by acute A viral hepatitis in a patient with Dubin-Johnson syndrome.

Keyword

Jaundice, chronic idiopathic; Hepatitis A; Cholestasis; Hyperbilirubinemia

MeSH Terms

Acute Disease
Adult
Bilirubin/blood
Cholangiopancreatography, Endoscopic Retrograde
Cholestasis/*diagnosis/etiology
Hepatitis A/complications/*diagnosis
Humans
Jaundice, Chronic Idiopathic/complications/*diagnosis
Liver/pathology
Male
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Microscopically, hepatocytes contained diffuse intracellular deposits of brown pigment (arrows) without evidence of cell damage in liver biopsy in 1995 (H&E, ×200).

• 

  Fig. 2 (A) Liver computed tomography and (B) endoscopic retrograde cholangiopancreatography showed hepato- splenomegaly and no significant abnormal finding in the biliary tract. Those examinations were performed in acute phase of acute hepatitis A.

• 

  Fig. 3 The change of serum total and direct bilirubin levels of the patient.

Reference

1. Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in liver cells; a new clinicopathologic entity with a report of 12 cases. Medicine (Baltimore). 1954. 33:155–197.

2. Butt HR, Anderson VE, Foulk WT, Baggenstoss AH, Schoenfield LJ, Dickson ER. Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). II. Evaluation of a large family with the trait. Gastroenterology. 1966. 51:619–630.

3. 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. 1970. 39:549–567.

4. 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. 1999. 64:739–746.

5. Swartz HM, Sarna T, Varma RR. On the natural and excretion of the hepatic pigment in the Dubin-Johnson syndrome. Gastroenterology. 1979. 76:958–964.

6. Novikoff AB, Essner E, Quintana N. Golgi Apparatus and Lysosomes. Fed Proc. 1964. 23:1010–1022.

7. Beker S, Read AE. Familial DubinJohnson syndrome. Gastroenterology. 1958. 35:387–389.

8. Hahn SS, Hahn YC, Hahn YS, Choi KW, Min BC, Kim OJ. A case of Dubin-Johnson syndrome. Taehan Uihak Hyophoe Chi. 1963. 6:609–614.

9. Berk PD. Bilirubin metabolism and the hereditary hyperbilirubinemias. Semin Liver Dis. 1994. 14:321–322.

10. Cohen L, Lewis C, Arias IM. Pregnancy, oral contraceptives, and chronic familial jaundice with predominantly conjugated hyperbilirubinemia (Dubin-Johnson syndrome). Gastroenterology. 1972. 62:1182–1190.

11. Koskelo P, Toivonen I, Adlercreutz H. Urinary coproporphyrin isomer distribution in the Dubin-Johnson syndrome. Clin Chem. 1967. 13:1006–1009.

12. Koff RS. Hepatitis A. Lancet. 1998. 351:1643–1649.

13. Ono S, Chida K, Takasu T. Guillain-Barré syndrome following fulminant viral hepatitis A. Intern Med. 1994. 33:799–801.

14. Mourani S, Dobbs SM, Genta RM, Tandon AK, Yoffe B. Hepatitis A virus-associated cholecystitis. Ann Intern Med. 1994. 120:398–400.

15. Inman RD, Hodge M, Johnston ME, Wright J, Heathcote J. Arthritis, vasculitis, and cryoglobulinemia associated with relapsing hepatitis A virus infection. Ann Intern Med. 1986. 105:700–703.

16. Gordon SC, Reddy KR, Schiff L, Schiff ER. Prolonged intrahepatic cholestasis secondary to acute hepatitis A. Ann Intern Med. 1984. 101:635–637.

17. Sciot R, Van Damme B, Desmet VJ. Cholestatic features in hepatitis A. J Hepatol. 1986. 3:172–181.

18. Schiff ER. Atypical clinical manifestations of hepatitis A. Vaccine. 1992. 10:Suppl 1. S18–S20.