Clinical Features Of Peutzjeghers Syndrome

Peutz-Jeghers syndrome is an autosomal dominantly inherited condition with an incidence of approximately 1 in 200,000 births.[2] This rare syndrome is characterized by hamartomatous polyposis of the GI tract, mucocuta-neous hyperpigmentation, and a significantly increased risk of developing intestinal and nonintestinal malignan cies. Symptoms attributed to PJS are often absent at the time of diagnosis, although patients can present early in life with gastrointestinal emergencies including intussusception, small bowel obstruction, and GI bleeding.[7] The hamartomatous polyps are located in the small bowel (particularly jejunum) in upward of 80% of patients, in the stomach in 40%, and in the colon or rectum in 40%.[1] Peutz-Jeghers syndrome polyps are composed of nonneoplastic tissue with a noticeably distorted architecture. Histologically, they are distinguished by normal epithelium covering frondlike projections that are composed of smooth muscle branching from the muscularis mucosa to the polyp head.[7] This pattern is often designated ''arborization.''

In addition to the characteristic hamartomas that occur in the gastrointestinal tract, pigmented macules along the vermillion border and in the interdigital spaces occur in individuals affected by PJS. Amplified numbers of melanocytes at the epidermal-dermal junction, containing increased basal-cell melanin, account for the blue to brown macules seen in PJS patients. Most commonly affected are the lips, the buccal mucosa, the skin around the eyes and nose, and the digits. The great majority of individuals with PJS (>95%) have evidence of increased melanin deposition. Although the macules on the lips and skin can fade over time, the buccal lesions typically persist.[8]

Getting Started With Dumbbells

Getting Started With Dumbbells

The use of dumbbells gives you a much more comprehensive strengthening effect because the workout engages your stabilizer muscles, in addition to the muscle you may be pin-pointing. Without all of the belts and artificial stabilizers of a machine, you also engage your core muscles, which are your body's natural stabilizers.

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