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KMID : 0614619940260030592
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Korean Journal of Gastroenterology
1994 Volume.26 No. 3 p.592 ~ p.595
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A Case of Rotor's Syndrome
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서영조
양창호/김문범/한상영/최석렬/김덕규
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Abstract
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Rotor's syndrome is a rare hereditary disorder characterized by chronic, nonhemolytic and predominantly conjugated hyprbilirubinemia with normal hepatic histology. It resembles the Dubin-Johnson syndrome, the main difference being the absence of
brown
pigment in the liver cell. Recent studies demonstrated that these two syndromes are distinct pathophysiologic entities.
Authors traced a 19 year-old male patient who had shown persistent and predominantly conjugated hyperbilirubinemia from birth. Urinary excretion of total coproporphyrin was markedly increased. Oral cholecystography visualized the gallbladder, but
99mTc-DISIDA scan showed markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tree. Histology of the liver was normal. So we confirmed the diagnosis of Rotor's syndrome.(Korean J Gastroenterol 1994 ; 26 :
592-595).
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KEYWORD
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