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Gastrointestinal diseases and their oro-dental manifestations: Part 4: Peutz-Jeghers syndrome.
Korsse, S E; van Leerdam, M E; Dekker, E.
Afiliación
  • Korsse SE; Department of Gastroenterology &Hepatology, HagaZiekenhuis, The Hague, the Netherlands.
  • van Leerdam ME; Department of Department of Gastroenterology &Hepatology, National Cancer Institute, Amsterdam, the Netherlands.
  • Dekker E; Department of Gastroenterology &Hepatology, Academic Medical Centre, Amsterdam, the Netherlands.
Br Dent J ; 222(3): 214-217, 2017 Feb 10.
Article en En | MEDLINE | ID: mdl-28184053
ABSTRACT
Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant inherited disorder, caused by germline mutations in the LKB1 tumour suppressor gene. It is clinically characterised by distinct perioral mucocutaneous pigmentations, gastrointestinal 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. Furthermore, patients have an increased risk for developing cancer, both in the gastrointestinal tract as in other organs. The medical management of PJS mainly consists of surveillance and treatment of the hamartomatous polyps. Upper and lower endoscopies are recommended for surveillance and removal of PJS polyps in the stomach and the small and large intestine. Furthermore, the high risk for pancreatic cancer justifies surveillance of the pancreatic region by MRI or endoscopic ultrasound. In addition, breast and gynaecological surveillance is recommended for female patients. Although the genetic defect underlying PJS is known, the pathogenesis of hamartomas and carcinomas is unclear. More insight into the molecular background of PJS might lead to targeted therapies for patients with this syndrome.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Peutz-Jeghers / Enfermedades Estomatognáticas / Enfermedades Gastrointestinales Tipo de estudio: Diagnostic_studies Límite: Humans Idioma: En Revista: Br Dent J Año: 2017 Tipo del documento: Article País de afiliación: Países Bajos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Peutz-Jeghers / Enfermedades Estomatognáticas / Enfermedades Gastrointestinales Tipo de estudio: Diagnostic_studies Límite: Humans Idioma: En Revista: Br Dent J Año: 2017 Tipo del documento: Article País de afiliación: Países Bajos