Epidemiology

DJS is usually diagnosed after puberty, although cases have been reported in neonates.[12][13][14][15] It is generally diagnosed between the ages of 10 and 30 years.[6] Overall, the prevalence is extremely low but more common than Rotor's syndrome, which is also an inherited disorder of conjugated hyperbilirubinaemia. No accurate prevalence figures are available. DJS has been described worldwide in all races, nationalities, and ethnic backgrounds, and in both sexes, although males predominate and present at an earlier age. The highest recognised prevalence (1 in 1300) is in Iranian Jews and is clustered in families. The prevalence in Moroccan Jews is nearly as high, reflecting the divergence of these populations 2000 to 2500 years ago.[16][17][18]

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