Recurrent small intestine intussusception in a patient with Peutz-Jeghers syndrome.

نویسندگان

  • Orestis Ioannidis
  • Styliani Papaemmanouil
  • George Paraskevas
  • Anastasios Kotronis
  • Stavros Chatzopoulos
  • Athina Konstantara
  • Nikolaos Papadimitriou
  • Apostolos Makrantonakis
  • Emmanouil Kakoutis
چکیده

Peutz-Jeghers syndrome is a rare hereditary autosomal dominant disease caused by a mutation of the tumor suppressor gene serine/threonine kinase 11 located in chromosome 19p13.3. It is characterized by the presence of extensive mucocutaneous pigmentation, especially of the lips and the occurrence of hamartomatous polyps throughout the gastrointestinal tract. Gastrointestinal hamartomas occur predominantly in the small intestine and can become symptomatic leading usually to intestinal obstruction and abdominal pain.We present a case of recurrent intestinal obstruction caused by small bowel intussusception treated by reduction, enterotomy and polypectomy and followed by intraoperative enteroscopy and endoscopic polypectomy.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Jejunoduodenal intussusception caused by a solitary polyp in a woman with Peutz-Jeghers syndrome: a case report

INTRODUCTION Peutz-Jeghers syndrome is a rare autosomal dominant disorder characterized by hamartomatous polyps and characteristic mucocutaneous pigmentation. The hamartomatous polyps of Peutz-Jeghers syndrome can cause intestinal occlusion, especially in the small intestine. Intussusception is seen frequently in children, but rarely in adults. CASE PRESENTATION We present the case of a 21-ye...

متن کامل

Unusual presentation of intussusception of the small bowel with peutz jeghers syndrome: report of a case.

The Peutz Jeghers syndrome (PJS) is an autosomal dominant disorder which is characterised by hamartomatous polyposes of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. We are reporting a case of a 15-year-old male with Peutz Jeghers syndrome, who presented to us with features of chronic intestinal obstruction and anaemia. Init...

متن کامل

Medical management update: Peutz Jeghers syndrome.

Peutz Jeghers syndrome (PJS) is an autosomal dominant disease characterized by hamartomatous polyposis and distinct mucocutaneous pigmentation. PJS is associated with an increased risk for several cancers and other complications such as small intestine intussusception, short bowel syndrome, and anemia. Medical management mainly consists of treatment of the polyps and surveillance. This medical ...

متن کامل

Peutz-Jeghers syndrome: an unusual cause of recurrent intussusception in a 7-year-old boy.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous pigmentations. Main symptoms of PJS in childhood are abdominal pain, obstruction, intussusception, and bleeding from hamartomatous polyps. PJS carries a high risk of gastrointestinal cancer with advancing years. Although, intussusception has...

متن کامل

Peutz-Jeghers syndrome with germline mutation of STK11

Peutz-Jeghers syndrome (PJS), also known as periorificial lentiginosis, is a rare autosomal dominant inherited disease with an incidence of 1/200,000 live-borns. Mutations in the serine-threonine kinase 11 (STK11) gene are considered the major cause of PJS. The most frequent complication at young age is recurrent intussusception due to multiple hamartomatous polyps, primarily in the small intes...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Revista espanola de enfermedades digestivas : organo oficial de la Sociedad Espanola de Patologia Digestiva

دوره 104 1  شماره 

صفحات  -

تاریخ انتشار 2012