MEHMET ALİ ERDOĞAN, Yahya ATAYAN, Yasir Furkan ÇAĞIN, Yılmaz BİLGİÇ, Murat ALADAĞ, Murat HARPUTLUOĞLU, Yüksel SEÇKİN, Aysen BAYRAC, Emine ŞAMDANCI

Steatohepatitis Coexisting with Dubin Johnson Syndrome: A Case Report

Dubin-Johnson syndrome (DJS) is a benign autosomal recessive liver disorder characterised by an intermittent jaundice caused by chronic and conjugated hyperbilirubinaemia. Many mutations in multidrug resistance associated protein (MRP-2) gene have been identified in patients with DJS. Although the disease is usually asymptomatic, some patients may experience vague abdominal pain. In this report, we present a patient with steatohepatitis diagnosed as DJS through a liver biopsy. The patient, 38 years of age, male was admitted to our clinic with vague abdominal pain and jaundice. High levels of serum transaminase, triglyceride, cholesterol and bilirubin were found. Ultrasonography showed Grade II hepatosteatosis in the liver. When steatohepatitis and intracytoplasmic dPAS positive pigment which is more prominent in the perivenular region (zone 3) was observed in the liver biopsy, the patient was diagnosed as DJS coexisting with steatohepatitis.

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

2. Iyanagi T, Emi Y, Ikushiro S. Biochemical and molecular aspects of genetic disorders of bilirubin metabolism. Biochim Biophys Acta. 1998;1407(3):173-84.

3. Angulo P, Lindor KD. Non-alcoholic fatty liver disease: Quadrennial review. The Journal of Gastroenterology and Hepatology. 2002;17 Suppl:186-90.

4. Druseja A, Nanda M, Das A, Das R, Bhansali A, Chawla Y. Prevalance of obesity, diabetes mellitus and hyperlipidaemia in patients with cryptogenic liver cirrhosis. Trop Gastroenterol. 2004;25(1):15-7.

5. Bayan K, Tüzün Y, Özcan M, Yılmaz Ş, Turgutalp S. Dubin-Johnson Sendromu tanili bir olgu nedeniyle konjuge hiperbilirubinemiler. Dicle Tıp Dergisi. 2008;35(3):196-200.

6. Paulusma CC, Kool M, Bosma PJ, Scheffer GL, ter Borg F, Scheper RJ, Tytgat GN, Borst P, Baas F, Oude Elferink RP. A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndrome. Hepatology 1997;25(6):1539-42.

7. Chowdhury JR, Wolkoff AW, Chowdhury NR, Arias IM. Hereditary jaundice and disorders of bilirubin metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds, The Metabolic and Molecular Bases of Inherited Disease. 7th edition Vol: II. New York: McGraw-Hill Inc, 1995;2161-208.

8. Swartz HM, Chen K, Roth JA. Further evidence that the pigment in the Dubin-Johnson syndrome is not melanin. Pigment Cell Res. 1987;1(2):69-75.

9. Frank M, Doss MO. Relevance of urinary coproporphyrin isomers in hereditary hyperbilirubinemias. Clin Biochem. 1989;22(3):221-2.

10. Li P, Wang Y, Zhang J, Geng M, Li Z. Dubin-Johnson syndrome with multiple liver cavernous hemangiomas: report of a familial case. Int J Clin Exp Pathol. 2013;6(11):2636-9.

11. Sanyal AJ, Campbell-Sargent C, Mirshahi F, Rizzo WB, Contos MJ, Sterling RK, Luketic VA, Shiffman ML, Clore JN. Nonalcoholic steatohepatitis: association of insulin resistance and mitochondrial abnormalities. Gastroenterology. 2001;120(5):1183-92.