Peutz-Jeghers syndrome diagnosed in childhood

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the development of benign hamartomatous polypus in the gastrointestinal tract with mucosal pigmenta- tion of the lips, gingiva, hard palate and around the eyes and nose. In 1921 Peutz was the first who recognized the familial association of gastrointestinal polyp. The original family was described by Jeghers in 1949. This syndrome is rare with a prevalence of about 1/50000. If a parent has PJS, there is a 50 percent chance that the abnormal gene will be passed to the child (chromosome 19). Almost 50 percent of patients develop cancer…