Mehmet Ali Erdogan, Yahya Atayan, Yasir Furkan Cagin, Yilmaz Bilgic, Murat

-
Anahtar Kelimeler:

-

Steatohepatitis Coexisting with Dubin Johnson Syndrome: A Case Report

AbstractDubin-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.  Key Words: Dubin-Johnson syndrome, steatohepatitis, hyperbilirubinaemia

___

  • Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome--a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006;49(4):500-4.
  • Iyanagi T, Emi Y, Ikushiro S. Biochemical and molecular aspects of genetic disorders of bilirubin metabolism. Biochim Biophys Acta. 1998;1407(3):173-84.
  • Angulo P, Lindor KD. Non-alcoholic fatty liver disease: Quadrennial review. The Journal of Gastroenterology and Hepatology. 2002;17 Suppl:186-90.
  • 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.
  • 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.
  • 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.
  • 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.
  • 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.
  • Frank M, Doss MO. Relevance of urinary coproporphyrin isomers in hereditary hyperbilirubinemias. Clin Biochem. 1989;22(3):221-2.
  • 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.
  • 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.
Medicine Science-Cover
  • ISSN: 2147-0634
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 2012
  • Yayıncı: Effect Publishing Agency ( EPA )
Sayıdaki Diğer Makaleler

The Acute Physiology and Chronic Health Evaluation (APACHE II) is a beneficial tool for

Cemil COLAK, Mustafa Said AYDOGAN, Ahmet Kadir ARSLAN, Aytac YUCEL

Department of Oral and Maxillofacial surgery, Amala Institute of Medical Sciences, Amala

A.n.c JOHN

Investigation of Pelvic Floor Muscle Strength of the Women Admitted to the Gynecology Clinics and the Evaluation of Affecting Factors [Malatya İl Merkezinde Jinekoloji Polikliniklerine Başvuran Kadınların Pelvik Taban Kas Kuvvetinin Değerlendirilmesi ve E

Filiz Ciledag OZDEMİR, Erkan PEHLİVAN

Mehmet Ali Erdogan, Yahya Atayan, Yasir Furkan Cagin, Yilmaz Bilgic, Murat

Mehmet Ali Erdogan, Yahya Atayan, Yasir Furkan Cagin, Yilmaz Bilgic, Murat Aladag, Murat Harputluoglu, Yuksel Seckin, Aysen Bayrac, Emine Samdancı

Afaki ve Göz İçi Lens Dislokasyonlu Olgularin Preoperatif Ultrason Biyomikroskopisi ile Değerlendirilmesi

Taner KAR, Yildiray YİLDİRİM, Cihan BUYUKAVSAR, Tuncay TOPAL, Eyup DUZGUN, Ali AYATA, Melih Hamdi UNA

-

Syed Mushtaq SHAH, Azhar MUSHTAQ, Hanief Mohamed DAR, Wasim QADİR

The Importance of Biological Samples and Toxicological Analysis in Suspected Munchausen by Proxy Syndrome (MbPS) Cases [Munchausen by Proxy Sendromu (MbPS) Şüphesi olgularında Biyolojik Örneklerin ve Toksikolojik Analizin Önemi]

Zeynep TURKMEN, Selda MERCAN, Can CALİCİ, Serkan TURKDOGRU, Neylan ZİYALAR

Investigation of Reasons for Age Assessment Demands among Cases Who Appear to Be in Chıldren Age Bracket According to Their Birth Records: 6.5 Years of Experience Nüfus Kaydına Göre Çocuk Yaş Grubunda Görünen Olguların Yaş Tayini Raporu İstem Nedenlerini

Hulya Guler, Ahsen Kaya, Orhan Meral, Nihal Erdogan, Suheyla Erturk

Effect of Adding Dexamethasone to Bupivacaine on Transversus Abdominis Plane Block [Transversus Abdominis Plane Bloğa Eklenen Deksametazonun Etkisi]

Nurçin GÜLHAŞ, Gülay KAYHAN, Mukadder ŞANLI, Arzu KITLIK, Mahmut DURMUŞ

Yahya Atayan, Yasir Furkan Cagın, Mehmet Ali Erdogan, Yılmaz Bilgic, Remzi 1, Yasir Furkan Cagın1, Mehmet Ali Erdogan1, Yılmaz Bilgic1, Remzi

Yahya Atayan, Yasir Furkan Cagın, Mehmet Ali Erdogan, Yılmaz Bilgic, Remzi Bestas, Murat Aladag