ABSTRACT
Dubin-Johnson syndrome (DJS) is a rare benign chronic disorder of bilirubin metabolism, characterized by conjugated hyperbilirubinemia, darkly pigmented liver and presence of abnormal pigment in hepatic parenchymal cells. This is a retrospective study of twenty cases of DJS highlighting their major clinical and pathological findings. Liver biopsies were available in all the cases, obtained during a fourteen-year period (January 1991 to March 2005). The patients' age ranged from 7-63 years (median 21 years). These twenty cases comprised 13 males and 7 females. Major clinical manifestations were recurrent or persistent jaundice, abdominal pain and fever. Duration of illness ranged from 9 months to 58 years (median 10 years). All of them had conjugated hyberbilirubinemia and total serum bilirubin levels ranged between 1.4-13 mg/dl (mean 4.4 mg/dl). Liver biopsies revealed presence of coarse granular brown pigment in the cytoplasm of hepatocytes more concentrated in the pericanalicular region and more prominent in centrilobular hepatocytes. Associated findings were presence of hepatitis B virus related chronic hepatitis (1), history of tubercular lymphadenitis (1), chronic cholecystitis in (2), coronary heart disease (1) and exacerbation during pregnancy (1).
Subject(s)
Adolescent , Adult , Biopsy , Child , Female , Hepatocytes/metabolism , Humans , Hyperbilirubinemia , Jaundice, Chronic Idiopathic/pathology , Liver/pathology , Liver Diseases/pathology , Male , Middle Aged , PigmentationABSTRACT
Se presentan 2 casos de síndrome de Dubin-Johnson, hermanos, un varón y una hembra, de 16 y 19 años, respectivamente. Ambos tuvieron el antecedente de ictericia recidivante sin diagnóstico definitivo. El varón fur remitido a nuestro centro por ictericia de 4 meses de evolución y la hembra con historia de ictericia en varias ocasiones durante los últimos 3 años, estudiada a partir del diagnóstico del hermano. En los 2 casos la laparoscopia y la biopsia hapática fueron diagnósticas y se obtuvieron muestras de tejido hepático para estudio ultraestructural. No existió asociación con litiasis vesicular. En el varón se observó además ceruloplasmina baja, aumento del cobre en tejido hepático fresco y de la excreción de cobre en orina, a pesar de que este síndrome no se asocia a otras hepatopatías. La ultraestructura del hígado mostró numerosos gránulos densos de pigmentos relacionados con los lisosomas y disminución o pérdida de vellosidades
Subject(s)
Humans , Male , Female , Adolescent , Adult , Biopsy , Liver/ultrastructure , Jaundice, Chronic Idiopathic/diagnosis , Laparoscopy , Jaundice, Chronic Idiopathic/pathologyABSTRACT
Relata-se um caso de síndrome de Dubin-Johnson com história de icterícia desde o nascimento, cujo diagnóstico foi confirmado pela presença do pigmento à microscopia hepática. Faz-se uma revisäo desta síndrome e procura-se compará-la a outras icterícias congênitas