Waqar Hussain, Dilshad Qureshi, Sajid Maqbool.
Dubin Johnson Syndrome.
Pak Paed J Jan ;21(1):43-4.

Six year old male child presented with jaundice and abdominal pain (off and on) for two months. He had the first episode on the third day of life without any signs of sepsis or blood group incompatibility. He had five to six similar episodes afterwards at variable times. At these times there were complaints of passage of dark urine and clay coloured stools, but there was no history of pruritis. He was the fourth issue of consanguineous marriage and no other family member was effected. On examination in addition to jaundice liver was palpable 1cm below the costal margin in the midclavicular line, firm and nontender. No other sign of chronic liver disease or splenomegaly was found. On laboratory investigations, bilirubin 1.6 mg/dl (1mg conjugated). Liver function tests and PT. APTT normal. Ultrasound showed marginally enlarged liver. On liver biopsy there was well maintained lobulai architecture and portal tracts. Hepatocytes were enlarged and revealed ballooning degeneration and were filled with granular brownish pigment. Iron staining was negative and PAS consistent with Dubin Johnson Syndrome.

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