%0 Journal Article
%T peutz-jeghers syndrome as a cause of intussusception
%A Durango- Guevara Kary
%A Arias-D赤az Antonio
%A Redondo-De Oro Katherine
%A Niˋo-Hern芍ndez Luc赤a Mercedes
%J Revista Ciencias Biom谷dicas
%D 2012
%I Universidad de Cartagena
%X Introduction: Peutz-Jeghers syndrome, also known as periorificial lentigines is adisease of autosomal dominant transmission. It is characterized by the associationof gastrointestinal polyposis and mucocutaneous pigmentation, which is evidentfrom the first years of life and may remain until adulthood. The polyps arehamartomatous and occur most frequently in the small intestine, although therearereported cases in the stomach and intestine.Case report: A 14 year old boy who is diagnosed with Peutz Jeghers consulted theemergency room at Napoleon Franco Pareja Children＊s Hospital, Cartagena, Colombia.He was admitted with acute abdominal pain secondary to intussusception thatrequired surgical repair and bowel resection. Delayed diagnosis, despite having lesionsin the oral mucosa and palmar and plantar skin since early childhood.We report this case to expand the general knowledge about this disease, seekingto increase the sensitivity of the medical staff for early diagnosis, thus avoidingthe morbidity and mortality.Conclusions: The Peutz-Jeghers syndrome has been associated with an increasedrisk of intussusception as in the case presented. It is also associated with anemia andcancer. Early diagnosis of the syndrome is important in order to search for polyps, identifyand resect them helps reduce the risk of intussusception.RESUMEN:Introducci車n: el s赤ndrome de Peutz-Jeghers, tambi谷n conocido como lentiginosisperiorificial es una enfermedad de transmisi車n autos車mica dominante. Se caracterizapor la asociaci車n de poliposis gastrointestinal y pigmentaci車n mucocut芍nea, la cual seevidencia desde los primeros a os de vida y puede permanecer hasta la edad adulta.Los p車lipos son de tipo hamartomatoso y se presentan con mayor frecuencia a niveldel intestino delgado aunque existen casos reportados en estomago e intestino grueso.Caso cl赤nico: joven de 14 a os que se le diagnostica s赤ndrome de Peutz Jeghersal consultar al servicio de urgencias del Hospital Infantil Napole車n Franco Pareja,Cartagena, Colombia. Ingres車 con dolor abdominal agudo secundario a invaginaci車nintestinal, que requiri車 correcci車n quir迆rgica y resecci車n intestinal. Diagn車stico tard赤o,a pesar de presentar lesiones en la mucosa oral y palmo plantares desde etapastempranas de la infancia. Se reporta este caso para ampliar el conocimiento generalsobre esta patolog赤a, buscando aumentar la sensibilidad del personal m谷dico para sudiagn車stico temprano, evitando as赤 la morbilidad y mortalidad que traen consigo suscomplicaciones.Conclusiones: el s赤ndrome de Peutz-Jeghers ha sido asociado a un incremento enel riesg
%K Peutz-Jeghers Syndrome
%K Intestinal Polyps
%K Periorificial Lentiginosis Syndrome.
%U http://www.revistacienciasbiomedicas.com/index.php/revciencbiomed/article/view/183/148