Epidemiology
The exact prevalence of this rare and inherited condition is not known. Most patients are asymptomatic and the condition may be diagnosed on the basis of incidental findings following investigations for other reasons. It was initially described by Rotor et al in the Philippines in 1948. Since then, additional cases have been reported in several countries, including the US, France, Japan, Mexico, Italy, and Papua New Guinea; its distribution is therefore worldwide. RS is the second rarest of the hereditary hyperbilirubinaemias; the first is Crigler-Najjar type I.[4] RS manifests no sex predisposition, and family studies suggest an autosomal-recessive mode of inheritance.[1][2][5] While the disease tends to present in adolescence or early adult life, cases have been reported in infants and young children.[6][7]
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