Epidemiology
Peutz-Jeghers syndrome (PJS) is rare. The reported incidence is 1 in 8300 to 1 in 200,000 live births.[1][2][3] One nationwide Danish study found an estimated prevalence of PJS of 0.6 per 100,000 persons.[4] Males and females are equally affected. While there is not thought to be a racial or ethnic predilection, a nationwide epidemiologic survey in Japan found a lower incidence at 0.07 per 100,000 persons.[5]
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