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A case of Dubin-Johnson syndrome in 10-year-old Korean male child was presented.
The presenting symptom was dark-colored urine for several years. Laboratory findings showed
increased bilirubin, especially direct-reacting bilirubin, and abnormal urinary coproporphyrin pattern (coproporphyrin I : 58%, coproporphyrin JK : 42%). Needle biopsy specimen of liver showed dark-brownish pigmentation in liver cells but was otherwise normal histologically.
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