Dabina-Johnson syndrome: causes, symptoms, diagnosis, treatment

The Dubin-Johnson syndrome is a chronic benign disease manifested by unstable jaundice with an increase in the level of predominantly conjugated bilirubin and bilirubinuria. It is inherited by an autosomal recessive type, distributed mainly in the Middle East among Iranian Jews.

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Causes of Dabin-Johnson syndrome

At the heart of the Dabin-Johnson syndrome (family chronic idiopathic jaundice with an unidentified pigment in the hepatic cells) is a congenital defect in the violation of the excretory function of hepatocytes (postmicrosomal hepatocellular jaundice). In this case, excretion of bilirubin from hepatocytes is violated, which leads to regurgitation of the quoted bilirubin into the blood (conjugated hyperbilirubinemia develops). Along with the defect of excretion of bilirubin, there is a violation of excretion of bromsulfalein, Bengal pink, cholecystografic agents. Excretion of bile acids is not impaired.

Characteristic of the Dabin-Johnson syndrome is the deposition of a significant amount of dark brown pigment in the cytoplasm of the hepatocytes. Macroscopically, the liver has a color from bluish-green to almost black. The nature of the pigment has not been finally established. AF Bluger (1984) assumes that melanin is its basis.

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Symptoms of Dabin-Johnson syndrome

The disease may first appear jaundice in pregnancy or on the background of taking oral contraceptives (both these conditions cause worsening of the excretory function of the liver). The forecast is favorable.

For Dabin-Johnson syndrome the following manifestations are characteristic:

• the disease is transmitted autosomal dominant way;
• more sick men;
• the first clinical signs of the disease are found in the period from birth to 25 years;
• the main symptom of the disease is chronic or intermittent jaundice, not clearly expressed;
• jaundice is accompanied by fatigue, nausea, decreased appetite, sometimes with abdominal pain; rarely - by unintentional itching;
• the content of bilirubin in the blood is increased to 20-50 μmol / l (rarely up to 80-90 μmol / l) due to a predominant increase in the conjugated (direct) fraction;
• bilirubinuria is observed; dark color of urine;
• the liver is not enlarged in most patients, occasionally its increase is 1-2 cm;
• bromosulfalein test, as well as radioisotope hepatology, reveal a sharp violation of the excretory function of the liver; other functional tests of the liver are not significantly altered;
• when radiologic examination of bile ducts is characterized by the absence of contrast or later and a weak filling of the biliary tract and bladder with contrast medium;
• there are no changes in blood indicators;
• the syndrome of cytolysis of hepatocytes is not typical.

The course of the Dabin-Johnson syndrome is chronic, favorable. Exacerbations of the disease are associated with intercurrent infections, physical overstrain, psychoemotional stress, alcohol intake, anabolic steroids, and operations. Often, Dabin-Johnson syndrome is complicated by the development of cholelithiasis.