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家族性粘膜皮肤色素沉着胃肠息肉病(Peutz-Jeghers Syndrome以下简称PJS)具有以下特点:①颊、唇的粘膜、牙龈、手足屈侧的皮肤有黑色素斑(简称黑斑);②胃肠道多发性息肉;③有家族遗传性。PJS的临床重要性在于胃肠息肉的并发症,它常引起腹痛、便血和缺铁性贫血等症状。肠套叠为本病的急性并发症,少数癌变。以往认为本病罕见,但McAllister等(1977)己收集欧美文献有320余例,青木花等(1976)收集日本文献共222例,刘汶(1984)收集我国文献共168例。我们从1972～1986共收治17例。以往文献主张治疗息肉要在发生肠套叠后急诊
Familial mucosal skin pigmentation and gastrointestinal polyposis (Peutz-Jeghers Syndrome referred to as PJS below) has the following characteristics: 1 mucous membranes of the cheeks and lips, gums, and flexed skin of the hands and feet (black spots); 2 gastrointestinal tract Multiple polyps; 3 family hereditary. The clinical importance of PJS lies in the complications of gastrointestinal polyps, which often cause abdominal pain, blood in the stool, and iron deficiency anemia. Intussusception is an acute complication of the disease, a small number of cancerous changes. In the past, this disease was considered to be rare, but McAllister et al. (1977) collected more than 320 cases of European and American literature, Aoki et al. (1976) collected 222 cases of Japanese literature, and Liu Wen (1984) collected 168 cases of Chinese literature. We received 17 cases from 1972 to 1986. Previous literature advocates that treatment of polyps should be performed after intussusception