![]() Peutz–Jeghers syndrome is rare and studies typically include only a small number of patients. It is inherited in an autosomal dominant pattern, which means that anyone who has PJS has a 50% chance of passing the disease on to their offspring. On chromosome 19, the gene known as STK11 ( LKB1) is a possible tumor suppressor gene. In 1998, a gene was found to be associated with the mutation. Anemia is also common due to gastrointestinal bleeding from the polyps. Ĭomplications associated with Peutz-Jeghers syndrome include obstruction and intussusception, which occur in up to 69 percent of patients, typically first between the ages of 6 and 18, though surveillance for them is controversial. The melanocytic macules are not associated with malignant transformation. Pigmented lesions are rarely present at birth, but often appear before 5 years of age. Dark blue, brown, and black pigmented mucocutaneous macules, are present in over 95 percent of individuals with Peutz-Jeghers syndrome. The first presentation is often bowel obstruction or intussuseption from the hamartomatous gastrointestinal polyps. The average age of first diagnosis is 23. Micrograph of Peutz-Jeghers type colonic polyp. ĭue to the increased risk of malignancies, direct surveillance is recommended. Specifically, it is associated with an increased risk of sex-cord stromal tumor with annular tubules in the ovaries. Patients with the syndrome also have an increased risk of developing carcinomas of the liver, lungs, breast, ovaries, uterus, testes, and other organs. Colorectal is the most common malignancy, with a lifetime risk of 39 percent, followed by breast cancer in females with a lifetime risk of 32 to 54 percent. The risks associated with this syndrome include a substantial risk of cancer, especially of the breast and gastrointestinal tracts. It has an incidence of approximately 1 in 25,000 to 300,000 births. This syndrome can be classed as one of various hereditary intestinal polyposis syndromes and one of various hamartomatous polyposis syndromes. In the context of bowel ischemia in particular ischemic colitis, the splenic flexure is sometimes referred to as Griffith's point, along with the upper rectum ( Sudeck's point).Peutz–Jeghers syndrome (often abbreviated PJS) is an autosomal dominant genetic disorder characterized by the development of benign hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on the lips and oral mucosa ( melanosis). The splenic flexure is a watershed region as it receives dual blood supply from the terminal branches of the superior mesenteric artery and the inferior mesenteric artery, thus making it prone to ischemic damage in cases of low blood pressure because it does not have its own primary source of blood. Splenic flexure syndrome is often found in those with irritable bowel syndrome (IBS), and is considered by some practitioners to be a type of IBS since it can also result from stress. Gas can build up at this flexure and give abdominal pain giving rise to a condition known as splenic flexure syndrome. The splenic flexure is the last and highest positioned flexure in the colon. The splenic flexure receives dual blood supply from the terminal branches of the superior mesenteric artery and the inferior mesenteric artery. The left colic flexure or splenic flexure (as it is close to the spleen) is the sharp bend between the transverse colon and the descending colon. ![]() It receives blood supply from the superior mesenteric artery. The hepatic flexure lies in the right upper quadrant of the human abdomen. ![]() The right colic flexure or hepatic flexure (as it is next to the liver) is the sharp bend between the ascending colon and the transverse colon. Note that "right" refers to the patient's anatomical right, which may be depicted on the left of a diagram. The right colic flexure is also known as the hepatic flexure, and the left colic flexure is also known as the splenic flexure. In the anatomy of the human digestive tract, there are two colic flexures, or curvatures in the transverse colon. ![]()
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