Peutz-Jeghers Syndrome and Cancer Linked by LKB1

Susanne Korsse @ Erasmus MC / Erasmus University Rotterdam

Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant inherited disorder, first described by the Dutch physician Jan Peutz in 1921. It is clinically characterized by mucocutaneous pigmentations, gastrointestinal (GI) polyposis and an increased cancer risk in adult life. Hamartomatous polyps can develop already in the first decade of life and may cause various complications, including abdominal pain, bleeding, anaemia, and acute intestinal obstruction. Cancer can develop at a later age, both in the GI tract as in other organs. The medical management of PJS mainly consists of surveillance, firstly to detect and remove hamartomas, and secondly to detect cancer at an early stage.

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