Dubin-Johnson syndrome is a rare, inherited disorder that affects the liver's ability to process and secrete bilirubin, resulting in chronic jaundice. It is caused by a genetic mutation in the ABCC2 gene that impairs the transport of conjugated bilirubin from liver cells into bile. The Bilirubin, Direct test is the most important test for diagnosis because it reveals the characteristic elevation of conjugated bilirubin that defines this condition.
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Dubin-Johnson syndrome is caused by a genetic mutation in the ABCC2 gene, which is inherited in an autosomal recessive pattern. This mutation affects the production or function of a protein called MRP2 (multidrug resistance-associated protein 2), which is responsible for transporting conjugated bilirubin from liver cells into the bile ducts. When this protein does not work properly, conjugated bilirubin accumulates in the liver and backs up into the bloodstream, causing the characteristic yellow discoloration of the skin and eyes known as jaundice.
The Bilirubin, Direct test is the most important test for Dubin-Johnson syndrome because it specifically measures conjugated bilirubin levels in the blood, which become significantly elevated in this condition. People with Dubin-Johnson syndrome show a characteristic pattern where direct bilirubin is disproportionately high compared to indirect bilirubin. The Bilirubin, Total test is also essential as a companion test because it measures the combined levels of both direct and indirect bilirubin, helping doctors calculate the ratio and confirm that the elevation is primarily in the conjugated form. This distinctive pattern of elevated direct bilirubin with a high direct-to-total ratio helps distinguish Dubin-Johnson syndrome from other causes of jaundice.
You should get tested if you notice persistent yellowing of your skin or the whites of your eyes, especially if multiple family members have experienced similar symptoms. Testing is also recommended if you have unexplained episodes of jaundice that worsen during illness, pregnancy, or when taking certain medications, or if routine blood work shows elevated bilirubin levels without an obvious cause. Because Dubin-Johnson syndrome is genetic, testing is particularly important if you have a family history of chronic jaundice or if you are planning to have children and want to understand your carrier status.
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What this means
Your direct bilirubin level is elevated above the normal range, which may indicate a problem with bile flow from your liver or a blockage in your bile ducts. While this could be related to several conditions including liver inflammation or gallstones, it's important to investigate the cause, especially if you're experiencing jaundice symptoms.
Recommended actions
Stay well-hydrated by drinking plenty of water throughout the day
Avoid alcohol and limit fatty foods to reduce stress on your liver
Include liver-supporting foods like leafy greens, beets, and turmeric in your diet
Follow up with your doctor for additional testing to identify the underlying cause
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