[The Dubin-Johnson syndrome: case report and review of literature].
The Dubin-Johnson syndrome is a hereditary deficiency in the excretion ofconjugated bilirrubin by hepatocytes characterized by chronic hyperbilirubinemia, alteration in coproporphyrin metabolism, and intracellular deposition of a dark melanin-like pigment giving the liver a typical black cast. We report a 28-year-old male patient who presented conjunctival jaundice and conjugated-hyperbilirubinemia without no other alteration in hepatic biochemistry. The diagnosis of this syndrome was perfomed by using the low-risk methods of laparoscopy-facilitated hepatic biopsy and oral cholecystography In contrast, we avoided the classical Bromsulphalein test because of potential severe side effects. We stress here the current importance of these tests for confirming the diagnosis. By using this methodology, we were not able to quantify the isomeric profile of the urinary coproporphyrins nor 99mTc-HIDA cholescintigraphy. In conclusion, we confirm the utility of hepatic biopsy with the aid of laparoscopy and oral cholecystography for the diagnosis of the Dubin-Johnson syndrome on the basis of their effectiveness and relative lack of complications.
Bosia
José Daniel
JD
Servicio de Gastroenterología-Sección de Hepatología, Hospital Profesor Rodolfo Rossi, Ciudad de La Plata, Provincia de Buenos Aires, Argentina. bosia@hsi-networks.com.ar
D'Ascenzo
María Virginia
MV
Borzi
Silvia
S
Cozzi
Susana
S
Defelitto
Jorge Raúl
JR
Curciarello
José Oscar
JO
D000328:Adult; D003229:Conjunctival Diseases / Q000175:diagnosis; D003306:Coproporphyrins / Q000652:urine; D006801:Humans; D006933:Hyperbilirubinemia, Hereditary / Q000175:diagnosis*; D007566:Jaundice, Chronic Idiopathic / Q000175:diagnosis; D008099:Liver / Q000473:pathology; D008297:Male; D013577:Syndrome
D003306:Coproporphyrins