Peutz-Jeghers综合征的诊断和治疗

• 作 者: 尹伯约 尹乐康 党德胜 闫于悌 朱建新 孟祥中 裴可夫 张艾莉 杨晓东
• 机 构: 甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院,甘肃省人民医院 730000,730000,730000,730000,
• 出 处: 中国实用外科杂志
• 发表日期: 1994年04期
• 关键词: 黑色素斑 多发性息肉 色素沉着 遗传性疾病 瘤性息肉 临床主要症状 肠粘膜 综合报道 Hutchinson 褐色斑点

Peutz-Jeghers综合征(PJS)为一少见的遗传性疾病,1896年Hutchinson曾记载一对孪生姐妹在口周围均有黑色素斑点,1919年We-ber补充报告该姐妹之一,于20岁时因肠套叠手术后死亡。1921年Peutz报告一家7人中有5人患此病,1949年Jeghers综合报道31例后定名。PJS具有典型的色素沉着、胃肠道多发性息肉及遗传因素三大特征。仅有典型的色素斑或胃肠道错构瘤性息肉称为不完全性者。临床主要症状和息肉产生并发症所致腹痛、肠套叠、肠梗阻、恶变、出血等有密切关系。 1 粘膜、皮肤色素沉着几乎所有病例口唇及口周都有色素沉着,尤以下唇为多。颊粘膜、齿龈、软腭、舌、眼睑、耳周、鼻侧、手指、脚趾甚至肠粘膜也常有色素斑点。我们遇1例在颈部有散在褐色斑点沉着。

Peutz-Jeghers syndrome (PJS) is a rare genetic disorder. Hutchinson, in 1896, has documented a pair of twin sisters with melanin around the mouth. In 1919, We-ber supplemented one of the sisters, After intussusception surgery died. In 1921 Peutz reported that 5 out of 7 people had the disease, and Jeghers comprehensively reported 31 in 1949. PJS has typical pigmentation, multiple gastrointestinal polyps and genetic factors three characteristics. Only typical pigmented or gastrointestinal hamartomatous polyps are referred to as incomplete. The main clinical symptoms and complications caused by polyps abdominal pain, intussusception, intestinal obstruction, malignant transformation, bleeding and so on are closely related. 1 mucosa, skin pigmentation Almost all cases of pigmentation lip and mouth, especially the lower lip is more. Buccal mucosa, gums, soft palate, tongue, eyelids, ear, nasal, fingers, toes and even intestinal mucosa pigment spots are often. We encountered a case of scattered brown spots on the neck.