[Rotor syndrome: relevance of the determination of coproporphyrin isomers in the urine in comparison with intrahepatic (alcohol-induced) cholestasis].
Porphyrin isomer examinations have been performed in two patients with Rotor syndrome (RS), one patient with Gilbert-Meulengracht syndrome and 12 patients with alcohol toxic cholestasis. Under both conditions, cholestasis and RS, total urinary coproporphyrin excretion as well as coproporphyrin isomer I was relatively and absolutely increased. Despite the different degree of the increase of coproporphyrin isomer I excretion between RS (69 vs. 72%) and cholestasis (47% on average), there are single cases with a coproporphyrin isomer I portion around 60%. In such cases, the differential diagnosis is quite difficult, so that the diagnosis "Rotor syndrome" should never be gained by one distinct examination; it is a diagnosis performed by exclusion of other diseases.
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Abteilung für Klinische Biochemie im Fachbereich Humanmedizin, Universität Marburg.
Frank
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M
Merkel
H G
HG
Doss
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M
D000328:Adult; D000368:Aged; D000437:Alcoholism / Q000150:complications*; D001663:Bilirubin / Q000097:blood; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis*; D003306:Coproporphyrins / Q000652:urine*; D003937:Diagnosis, Differential; D006801:Humans; D007566:Jaundice, Chronic Idiopathic / Q000175:diagnosis* / Q000235:genetics; D008111:Liver Function Tests; D008297:Male; D011166:Porphyrins / Q000652:urine*
D003306:Coproporphyrins; D011166:Porphyrins; C026971:coproporphyrin III; C031467:coproporphyrin I; D001663:Bilirubin
Rotor-Syndrom: Relevanz der Koproporphyrin-Isomerenbestimmung im Urin im Vergleich zur intrahepatischen (alkoholtoxischen) Cholestase.