SciELO - Scientific Electronic Library Online

 
vol.71 número3Ventilación mecánica en recién nacidos con infección respiratoria aguda baja por virus respiratorio sincicial índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados

Revista

Articulo

Indicadores

Links relacionados

Compartir


Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

FERREIRO C., Myriam et al. Familial Peutz-Jeghers syndrome. Rev. chil. pediatr. [online]. 2000, vol.71, n.3, pp.214-219. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062000000300007.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous melanin deposits and hamartomatous polyps of the gastrointestinal tract. Intussusception and intestinal bleeding are the usual symptoms. An increased risk for gastrointestinal and non-gastrointestinal cancer have been described. We report a case of familiar PJS, the recently described mutation in the gene STKI 1 and the management of PJS, with emphasis on the endoscopic resection of the polyps on an outpatient basis. The first degree relatives of an index case should be followed up annually since birth. Once the diagnosis is certain the approach must be aggressive. Gastric and colonic polyps larger than 5mm and small bowel polyps larger than 15 mm should be removed. Early screening for cancer is crucial

Palabras clave : Peutz-Jeghers syndrome; polyps; cancer.

        · resumen en Español     · texto en Español

 

Creative Commons License Todo el contenido de esta revista, excepto dónde está identificado, está bajo una Licencia Creative Commons