Popis: |
The Peutz jeghers syndrome is a familial affection with dominant autosomic transmission characterized by a hamartoma digestive polyposis and a cutaneous mucous lentiginosis with periorifice predominance. This affection represented 3 into 10% for the familial polyps. The digestive polyps constitute the must important part of the pentz jeghers syndrome because they determined to only the clinic manifestation and the disease prognostic with there complications: intestinal invaginations, haemorrhage. In this work, we report two observations of patients presented a Peurtz Jeghers syndrome, and we determine, after literature review the epidemiologic, pathogenic, clinic, anatomy pathologic, prognosis and therapeutics of this effection. |