What is Dubin–Johnson Syndrome?
Dubin–Johnson Syndrome is a rare, inherited, benign liver disorder that presents with chronic jaundice. The condition is caused by impaired excretion of conjugated bilirubin from liver cells into the bile ducts due to a defect in a specific transport protein (MRP2).
A distinctive feature of this syndrome is the characteristic dark pigmentation of the liver (caused by pigment deposition), which can be observed during biopsy or surgical procedures. Despite this, the structure of the liver is usually preserved, cirrhosis does not develop, and the prognosis is favorable.
Persistent or intermittent jaundice (most often beginning in childhood or adolescence)
Sometimes: fatigue, abdominal discomfort, weakness
Absence of itching (unlike in cholestasis)
Dark urine, with stools usually of normal color
Overall health is generally good
Yes. It is inherited in an autosomal recessive manner. A child is affected only if they inherit one defective copy of the gene from each parent.
There is no specific treatment, since this syndrome does not damage the liver. Management is symptomatic:
Maintaining overall well-being and a healthy lifestyle
Avoiding factors that can worsen jaundice (stress, infections, hormonal changes, certain medications)
Regular follow-up with a gastroenterologist or hepatologist
It is a benign condition that does not shorten life expectancy. The only issue may be chronic jaundice, which can have cosmetic significance.
Blood tests: elevated conjugated bilirubin with normal liver enzyme levels
No evidence of liver damage (normal ALT, AST)
Liver biopsy: dark pigmentation of cells with a characteristic pigment
Genetic testing can confirm a mutation in the ABCC2 (MRP2) gene
The involvement is limited to the liver — dark pigmentation and impaired bilirubin transport. Other organs are not affected.
No cure is needed, since the condition does not cause complications.
Explain to your child that this is a safe condition and does not harm their health.
Avoid factors that may worsen jaundice (stress, prolonged infections, certain medications — for example, hormonal contraceptives later in life).
Your child has no restrictions on diet, learning, or physical activity.
Consult a doctor regularly for monitoring, although serious treatment will not be required.
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