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Dubin-Johnson syndrome with systemic lupus erythematosus: a case report
引用本文:Mamun-Al Mahtab,Md. Fazal Karim,Salimur Rahman,Abul Barkat Muhammad Adnan. Dubin-Johnson syndrome with systemic lupus erythematosus: a case report[J]. Hepatobiliary & Pancreatic Diseases International, 2006, 0(4)
作者姓名:Mamun-Al Mahtab  Md. Fazal Karim  Salimur Rahman  Abul Barkat Muhammad Adnan
作者单位:Department of Hepatology Bangabandhu Sheikh Mujib Medical University Dhaka Bangladesh,Department of Hepatology Bangabandhu Sheikh Mujib Medical University Dhaka Bangladesh,Department of Hepatology Bangabandhu Sheikh Mujib Medical University Dhaka Bangladesh,Department of Hepatology Bangabandhu Sheikh Mujib Medical University Dhaka Bangladesh
摘    要:Introduction Dubin-Johnson syndrome (DJS) is a rare recessively inherited conjugated hyperbilirubi- nemia caused by deficiency of the canalicular multi-drug resistance/multi-specific organic anionic transporter protein (MDR2/cMOAT). Thus bilirubin is conjugated but inefficiently secreted into bile, which results in accumulation of conjugated and, to some extent, unconjugated bilirubin in blood, leading to hyperbilirubinemia and bilirubinuria. But, the results of liver function tests are no…


Dubin-Johnson syndrome with systemic lupus erythematosus: a case report
Mamun-Al Mahtab,Md. Fazal Karim,Salimur Rahman and Abul Barkat Muhammad Adnan. Dubin-Johnson syndrome with systemic lupus erythematosus: a case report[J]. 国际肝胆胰疾病杂志, 2006, 0(4)
Authors:Mamun-Al Mahtab  Md. Fazal Karim  Salimur Rahman  Abul Barkat Muhammad Adnan
Affiliation:Mamun-Al Mahtab,Md. Fazal Karim,Salimur Rahman and Abul Barkat Muhammad Adnan Department of Hepatology,Bangabandhu Sheikh Mujib Medical University,Dhaka,Bangladesh
Abstract:BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.
Keywords:Dubin-Johnson syndrome  systemic lupus erythematosus  conjugated hyperbilirubinemia  autoimmune disorder  chance occurrence
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