What is Dubin Johnson?
Dubin-Johnson syndrome (DJS) is a disorder passed down through families (inherited). In this condition, you may have mild jaundice throughout life. The digestive system organs in the abdominal cavity include the liver, gallbladder, stomach, small intestine and large intestine.
What is the difference between Rotor syndrome and Dubin-Johnson syndrome?
Rotor type hyperbilirubinemia is a distinct yet similar disorder to Dubin–Johnson syndrome – both diseases cause an increase in conjugated bilirubin. Whereas rotor syndrome differs in that it is a result of impaired hepatocellular storage of conjugated bilirubin that leaks into plasma causing hyperbilirubinemia.
Why does Dubin Johnson have black liver?
The buildup of bilirubin in the body causes the yellowing of the skin and whites of the eyes in people with Dubin-Johnson syndrome. The black liver in affected individuals is due to a buildup of different substance normally transported out of the liver by the protein produced from the ABCC2 gene.
What is the defect in Dubin-Johnson syndrome?
The primary defect in Dubin-Johnson syndrome is a mutation in an apical canalicular membrane protein responsible for the excretion of bilirubin and other nonbile salt organic anions.
How is Dubin-Johnson syndrome diagnosed?
The diagnosis of Dubin-Johnson syndrome (DJS) should be considered in all individuals with elevated conjugated bilirubin levels with otherwise normal liver function test findings. The diagnosis can be confirmed by demonstrating an increase in the ratio of urinary coproporphyrin I to coproporphyrin III.
Why is Dubin Johnson benign?
General Discussion. Dubin Johnson syndrome (DJS) is a rare, benign genetic liver disorder. It is inherited in an autosomal recessive pattern and is characterized by buildup of bilirubin, which is normally excreted by the liver into the bile.
What enzyme is increased in Dubin Johnson and in Rotor syndrome?
Dubin-Johnson syndrome is associated with increased plasma conjugated bilirubin, typically with mild jaundice (total bilirubin, 2-5 mg/dL), and intense dark pigmentation of the liver due to accumulation of lipofuscin pigment, brown granules from liposomal degradation of lipids.
Is Dubin Johnson conjugated?
Dubin-Johnson syndrome is characterized by conjugated hyperbilirubinemia without other serum enzyme abnormalities. Patients with Dubin-Johnson syndrome have a normal life span. A black or brownish lysosomal pigment in the hepatocytes is a characteristic histologic feature.
What is Dubin?
Summary. Dubin Johnson syndrome (DJS) is a rare, benign genetic liver disorder. It is inherited in an autosomal recessive pattern and is characterized by buildup of bilirubin, which is normally excreted by the liver into the bile.
How is Dubin-Johnson syndrome (DJS) characterized?
A combination of intense and prolonged visualization of the liver following intravenous administration of the radiopharmaceutical dye, with delayed to no visualization of the gallbladder, is unique to Dubin-Johnson syndrome (DJS).
What is the life expectancy of someone with Dubin-Johnson syndrome?
Dubin-Johnson syndrome is a benign condition, and life expectancy among patients is normal. An interesting case report describes an infant who received a living related liver transplant donor graft from his mother, who had Dubin-Johnson syndrome.
How is Dubin-Johnson syndrome (DBS) treated?
Management. Dubin-Johnson syndrome is a benign disorder that requires no specific therapy, although patients should be warned that pregnancy, oral contraceptive use, and intercurrent illnesses can exacerbate the associated jaundice. In the past, patients were treated with phenobarbital, which was used primarily to reduce serum bilirubin levels.
Is a liver biopsy necessary for the diagnosis of Dubin-Johnson syndrome?
Although a liver biopsy is not necessary for the diagnosis of Dubin-Johnson syndrome, patients may be noted to have a dark liver during routine surgery (eg, cholecystectomy), prompting biopsy.