Dubin-Johnson Syndrome

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Dubin-Johnson Syndrome is a rare genetic disorder that affects the liver’s ability to process bilirubin, resulting in a buildup of this pigment in the body. This condition, also known as chronic idiopathic jaundice, is characterized by hyperbilirubinemia, dark urine, jaundice, hepatomegaly, fatigue, abdominal pain, nausea, vomiting, and pruritus. While there is no known cure for Dubin-Johnson Syndrome, there are ways to manage the symptoms and improve the quality of life for those affected.

Understanding Dubin-Johnson Syndrome

Dubin-Johnson Syndrome is a rare autosomal recessive disorder caused by mutations in the ABCC2 gene, which encodes a protein called multidrug resistance-associated protein 2 (MRP2). MRP2 is responsible for transporting bilirubin from the liver cells into the bile, allowing it to be excreted from the body. In individuals with Dubin-Johnson Syndrome, the function of MRP2 is impaired, leading to the accumulation of bilirubin in the liver and bloodstream.

Symptoms of Dubin-Johnson Syndrome

The most common symptom of Dubin-Johnson Syndrome is jaundice, which is characterized by yellowing of the skin and eyes. This occurs due to the buildup of bilirubin in the body. Other symptoms may include:

  • Dark urine: The excess bilirubin in the bloodstream is excreted through the kidneys, resulting in dark-colored urine.
  • Hepatomegaly: The liver may become enlarged due to the accumulation of bilirubin and other substances.
  • Fatigue: The buildup of bilirubin can affect liver function, leading to fatigue and weakness.
  • Abdominal pain: Some individuals with Dubin-Johnson Syndrome may experience abdominal discomfort or pain.
  • Nausea and vomiting: These symptoms may occur due to the impaired liver function.
  • Pruritus: Itching of the skin may be present, although it is less common.

Diagnosis and Treatment

Dubin-Johnson Syndrome is typically diagnosed through a combination of clinical evaluation, blood tests, and genetic testing. Blood tests can reveal elevated levels of bilirubin, while genetic testing can identify mutations in the ABCC2 gene.

While there is no cure for Dubin-Johnson Syndrome, treatment focuses on managing symptoms and preventing complications. This may involve:

  • Dietary modifications: A low-fat diet may be recommended to reduce the workload on the liver.
  • Avoiding certain medications: Some drugs can worsen liver function in individuals with Dubin-Johnson Syndrome, so it is important to avoid them.
  • Regular monitoring: Regular blood tests and liver function tests can help monitor the progression of the disease and detect any complications.
  • Symptom management: Medications may be prescribed to alleviate symptoms such as itching or abdominal pain.

Living with Dubin-Johnson Syndrome

While Dubin-Johnson Syndrome can be a lifelong condition, many individuals with the disorder lead normal, healthy lives. It is important to maintain a healthy lifestyle and follow the recommended treatment plan to manage symptoms effectively. Here are some tips for living with Dubin-Johnson Syndrome:

  • Stay hydrated: Drinking plenty of water can help flush out toxins and support liver function.
  • Eat a balanced diet: A diet rich in fruits, vegetables, and whole grains can provide essential nutrients and support overall health.
  • Avoid alcohol and smoking: These substances can further damage the liver and worsen symptoms.
  • Exercise regularly: Regular physical activity can help improve liver function and overall well-being.
  • Manage stress: Stress can exacerbate symptoms, so finding healthy ways to cope with stress is important.

It is also crucial for individuals with Dubin-Johnson Syndrome to communicate with their healthcare providers and seek regular medical care. This can help monitor the progression of the disease and address any concerns or complications that may arise.


Dubin-Johnson Syndrome is a rare genetic disorder that affects the liver’s ability to process bilirubin. While there is no cure for this condition, individuals with Dubin-Johnson Syndrome can lead normal lives by managing their symptoms and following a healthy lifestyle. Regular medical care, dietary modifications, and avoiding certain medications are essential for maintaining liver function and overall well-being. By understanding the symptoms and treatment options, individuals with Dubin-Johnson Syndrome can take control of their health and live fulfilling lives.

Haroon Rashid, MD
Rate author
Urgent Care Center of Arlington, VA
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