Pedestrian syndrome genetic intestinal.. Pigmentations on the oral mucosa. Intestinal polyposis in the small intestine

Pedestrian syndrome genetic intestinal Peutz Jeghars syndrome:

Hereditary disease is transmitted as an autosomal dominant trait.

Symptoms:

  • Pigmentations Qatamenih about the mouth and nose and eye (Tricircle - Triple circuit) small freckles can we also see in the soles of the hand and soles of the feet.
  • Pigmentations on the oral mucosa.
  • Intestinal polyposis in the small intestine.

Note: Boiler or polyp Polype:

  • Is Hawi epidermal proliferation gives cauliflower-shaped glands and be-and long lesions are.
  • In other words, is adenoma infects the small intestine or large intestine, which is not considered such as cancerous lesion, but symptoms may appear bowel obstruction, constipation and bleeding.
  • Differentiated from Gardner's syndrome that the latter where there polyposis in the large intestine and be pre-cancerous.

What is Gardner syndrome in oral pathology?

Gardner described a syndrome consisting of hereditary intestinal polyposis with osteomas and multiple cutaneous and subcutaneous lesions in 1953.

What is juvenile polyposis syndrome?

Juvenile polyposis syndrome (JPS) is an autosomal dominant condition characterized by multiple hamartomatous polyps throughout the gastrointestinal tract. Individuals with JPS are at increased risk for colorectal and gastric cancer [1,2].

Is Lynch syndrome a polyposis syndrome?

Lynch syndrome, also known as hereditary non-polyposis colorectal cancer (HNPCC), is the most common cause of hereditary colorectal (colon) cancer. Certain types of skin cancers.

Why is it called Gardner's syndrome?

Gardner syndrome is named for geneticist Eldon J. Gardner, who discovered it in 1951 while studying a large family in Utah whose members were thought to have FAP. They had numerous colon and rectal polyps, but they also had multiple fibromas, osteomas and cysts throughout their bodies.

What cancer is associated with Peutz-Jeghers syndrome?

Patients with Peutz-Jeghers syndrome are at an increased risk for developing GI cancers of the colorectal, pancreatic, and gastric systems, in addition to a wide variety of non-GI epithelial malignancies including breast, uterine, cervical, lung, ovarian, and testicular cancers.

What is the triad of Peutz-Jeghers syndrome?

Peutz-Jeghers syndrome, also known as intestinal polyposis II, is a familial condition characterized by the triad of (1) mucocutaneous pigmentation, (2) benign polyps occurring in any part of the intestinal tract but mainly in the jejunum, and (3) autosomal dominant inheritance.

What is the characteristic oral clinical feature of Peutz-Jeghers syndrome?

Children with Peutz-Jeghers syndrome often develop small, dark-colored spots on the lips , around and inside the mouth , near the eyes and nostrils, and around the anus. These spots may also occur on the hands and feet. They appear during childhood and often fade as the person gets older.

What is the reason of oral pigmentation in Peutz-Jeghers syndrome?

Peutz-Jeghers syndrome is an inherited condition, whose key features are mucocutaneous hyperpigmented macules in association with hamartomatous polyps in the gastrointestinal tract. The cause is a specific germline alteration (usually deletion) at the serine threonine kinase 11 (STK11) gene on chromosome 19.
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