A Case of Sustained Cholestasis Caused by Acute A Viral Hepatitis in Dubin-Johnson Syndrome / 대한소화기학회지

Dubin-Johnson syndrome is a rare clinical entity. It shows intermittent symptoms such as chronic or intermittent jaundice, abdominal pain, weakness, nausea, vomiting, anorexia and diarrhea. Symptoms are precipitated or aggravated by pregnancy, alcoholism, surgical procedures and intercurrent disease. Chronic idiopathic jaundice is typical of Dubin-Johnson syndrome and its prognosis is good. We describe a case of prolonged cholestasis for more than 10 months caused by acute A viral hepatitis in a patient with Dubin-Johnson syndrome. It is a first report of cholestasis complicated by acute A viral hepatitis in a patient with Dubin-Johnson syndrome.

Hepatectomía derecha extendida por colangiocarcinoma intrahepático en un paciente con síndrome de Dubin-Johnson / Hepatectomy for cholangiocarcinoma in one patient with Dubin Johnson syndrome

Dubin-Johnson syndrome (SDJ) is a rare benign autosomal recessive congenital disease that causes jaundice. We report a 71 years old male with a Dubin Jonson syndrome diagnosed at the age of 40. On a ultrasound examination and magnetic resonance imaging, a liver tumor was found, that was excised. The pathology report showed a cholangiocarcinoma. During the postoperative period, the patient had a hyperbilirubinemia that subsided slowly. There are reports of hyperbilirubinemia of difficult management after hepatectomy in patients with Dubin-Johnson syndrome.

El síndrome de Dubin-Johnson (SDJ) es una patología benigna, familiar, con carácter autosómico recesivo y de aparición poco frecuente. Consiste en un defecto en la excreción hepática de la bilirrubina conjugada que permite su entrada al canalículo biliar, manifestando un cuadro clínico de ictericia silente. La asociación de esta enfermedad con colangiocarcinoma intrahepático es excepcional, siendo difícil la sospecha precoz de este diagnóstico y también el manejo de la ictericia postoperatoria. El objetivo de este trabajo es describir un caso clínico con estas características y revisión de la literatura existente. Se presenta a un paciente de sexo masculino de 71 años de edad, con antecedente de síndrome de Dubin-Johnson diagnosticado a los 40 años. Asintomático, en exámenes de rutina se encuentra un tumor hepático en la ecotomografía abdominal, que luego se corrobora en la TAC y RNM, mide aproximadamente 10 cm de diámetro, de ubicación central y derecho. Resto de estudio no muestra diseminación. Se realiza laparoscopia diagnóstica y ecografía intraoperatoria confirmando lo visto por imágenes, y se decide convertir a laparotomía para efectuar hepatectomía derecha extendida. Biopsia rápida y diferida informan colangiocarcinoma. Evoluciona favorablemente en el postoperatorio, con hiperbilirrubinemia sostenida de lenta resolución. La asociación de hepatectomía con síndrome de Dubin-Johnson es de muy baja frecuencia, existiendo poca literatura al respecto. Se ha descrito que la hiperbilirrubinemia en el postoperatorio es de difícil manejo, sin embargo, en este caso el paciente evolucionó favorablemente sin necesidad de alguna terapia específica.

Association of Dubin-Johnson syndrome and portal vein thrombosis.

Dubin-Johnson syndrome is neither complicated by liver cell necrosis nor associated with portal hypertension. We report a 22-year-old man who had recurrent episodes of jaundice (conjugated hyperbilirubinemia) because of Dubin-Johnson syndrome and portal hypertension secondary to portal vein thrombosis. The relationship between Dubin-Johnson syndrome and portal vein thrombosis in this case is most likely a chance occurrence.

A Case of Idiopathic Fibrosing Pancreatitis

We experienced a case of chronic fibrosing pancreatitis in an 18/12-year-old girl, which was idiopathic because there were no familial back ground, no cystic fibrosis of pancrease, no ductal anomalies and obstruction. The patient presented intermittent colicky abdominal pain and progressive obstructive jaundice, but T-tube drainage and removal of the lymph nodes around the common bile duct relieved her symptoms and disease process. This seems to be the first case reported in a Korean child. Idiopathic fibrosing pancreatitis should be considered in the differential diagnosis of abdominal pain with obstructive jaundice in children.