Dabin-Johnson Syndrome

Dubin-Johnson syndrome

Dabin-Johnson syndrome is a rare hereditary disease characterized by impaired transport of bile pigments in hepatocytes.

Main symptoms:

  1. Increased levels of conjugated bilirubin in the blood, leading to jaundice.

  2. Dark urine due to high conjugated bilirubin content.

  3. Accumulation of black pigment in the epithelium of the salivary glands, renal tubules and sweat glands.

  4. Possible liver damage in the form of chronic hepatitis.

  5. Increased risk of developing cholelithiasis.

Diagnosis is based on the clinical picture and biochemical blood test. Differential diagnosis is made with Gilbert's syndrome.

Treatment is symptomatic. The prognosis is generally favorable.

The disease is inherited in an autosomal recessive manner. It occurs extremely rarely, more often in men. First described in 1954 by American doctors Dubin and Johnson.



Dabin-Johnson-Syndrome: causes, treatment and possible complications

Dabin-Johnson syndrome is a hereditary metabolic disorder in the body that occurs due to a failure in the synthesis of mitochondrial proteins. At the moment, there is no accurate way to diagnose this disorder in the early stages and prevent its further progression. But with timely diagnosis, this pathology can be cured. One of the most effective methods is bone marrow transplantation. Thanks to this procedure you can achieve