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Sprinz-Nelson syndrome

Last updated June 22, 2020

Approved by: Krish Tangella MD, MBA, FCAP

Dubin-Johnson Syndrome (DJS) is a rare genetic disorder, which is characterized by mild jaundice that is present throughout the life of the individual.


The topic Sprinz-Nelson syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition Dubin-Johnson Syndrome.

Quick Summary:

  • Dubin-Johnson Syndrome (DJS) is a rare genetic disorder, which is characterized by mild jaundice that is present throughout the life of the individual
  • The basic defect in this condition is that the liver is not able to transport bilirubin out of it. Some individuals may have liver enlargement, liver tenderness, weakness, nausea or vomiting
  • Normally, bilirubin (a yellow chemical) is the main breakdown product of older red blood cells. It is then packaged in the liver (conjugation) and sent in the bile (digestive fluid that helps in fat digestion) to the gastrointestinal tract to be excreted in the feces. If there is either excess bilirubin or a disruption of the bilirubin pathway, it starts accumulating in the liver
  • Eventually, excess bilirubin in the liver, passes into the bloodstream thereby increasing bilirubin levels in blood, resulting in hyperbilirubinemia. Hyperbilirubinemia causes jaundice, which is yellowing of the white region (sclera) of eyes, skin, and nails
  • Jaundice can occur when one of the following pathways is affected:
    • There is too much breakdown of red blood cell
    • The liver cannot package bilirubin adequately
    • Obstruction of the flow of bile
    • Impaired entry of packaged bilirubin into bile; Dubin-Johnson Syndrome affects this pathway

  • Usually, there is no elevation of other liver enzymes, unlike with other liver diseases, and a majority of the individuals need no treatment. The prognosis of Dubin-Johnson Syndrome is generally good in most individuals

Please find comprehensive information on Dubin-Johnson Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: July 10, 2017
Last updated: June 22, 2020