摘要
消化道多发性息肉综合征(PJS)是一种以皮肤黏膜色素沉着、消化道息肉为主要临床表现的常染色体显性遗传性疾病。其病因可能与肝激酶B1(丝氨酸/苏氨酸激酶11)基因,脆性组氨酸三联体基因和p53基因密切相关。PJS除了消化道息肉可能发生恶变之外,还可伴发其他肿瘤。皮肤黏膜黑色素斑是PJS独有的特征,患者常常首诊于口腔科,其治疗方式有手术切除、冷冻、磨皮、电干燥和激光等,而息肉则以手术切除为主。PJS的癌变率较高,应尽早治疗并定期监测预防癌变。
Peutz-Jeghers syndrome（PJS） is autosomal dominant genetic disease. Mucosa pigmentation and gastrointestinal polyps are the main clinical manifestations in PJS patients. The etiology is complex and unclear and may be closely related with the liver kinase Bl, fragile histidine triad, and p53 genes. PJS gastrointestinal malignant polyps may form. In addition, PJS can also be associated with other tumors. Mucocutaneous melanin spots are unique characteristics of PJS in patients. Patients often receive treatment in stomatology departments, and treatment methods are surgery, freezing, dermabrasion, power dry, and laser treatments. Gastrointestinal polyps are removed by surgery. PJS patients should be treated as soon as possible because tumor susceptibility is higher than normal. PJS patients need to regularly monitor their physical condition to prevent cancer.
出处
《国际口腔医学杂志》
CAS
北大核心
2015年第5期597-599,共3页
International Journal of Stomatology
