The mealoncytic macules are not associated with malignant transformation.Complications 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. Anemia is also common due to gastrointestinal bleeding from the polyps. Genetics
In 1998, a gene was found to be associated with the mutation. On chromosome 19, the gene known as STK11 (LKB1) is a possible tumor suppressor gene. 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.Peutz–Jeghers syndrome is rare and studies typically include only a small number of patients. Even in those few studies that do contain a large number of patients, the quality of the evidence is limited due to pooling patients from many centers, selection bias (only patients with health problems coming from treatment are included), and historical bias (the patients reported are from a time before advances in the diagnosis of treatment of Peutz–Jeghers syndrome were made). Probably due to this limited evidence base, cancer risk estimates for Peutz–Jeghers syndrome vary from study to study. There is an estimated 18–21% risk of ovarian cancer, 9% risk of endometrial cancer, and 10% risk of cervical cancer, specifically adenoma malignum. Diagnosis
The main criteria for clinical diagnosis are:
Family history
Mucocutaneous lesions causing patches of hyperpigmentation in the mouth and on the hands and feet.
Telmisartan/amlodipine, sold under the brand name Twynsta among others, is a medication used to treat high blood pressure. It is a combination of telmisartan an angiotensin II receptor antagonist with amlodipine a calcium channel blocker. It is taken by mouth.Common side effects include dizziness, swelling, and back pain. Severe side effects may include low blood pressure, kidney problems, electrolyte problems, and a heart attack. Use during pregnancy may harm the baby. Telmisartan works by blocking the effects of angiotensin II while amlodipine works by decreasing calcium ion entry into smooth muscle and heart muscle.The combination was approved for medical use in the United States in 2009. The combination is on the World Health Organizations List of Essential Medicines. It is available as a generic medication. References
External links
"Amlodipine besylate mixture with telmisartan". Drug Information Portal. U.S. National Library of Medicine.