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黑斑息肉综合征(Peutz-Jeghers syndrome,PJS)是一种以皮肤黏膜色素沉着斑、胃肠道多发息肉、家族遗传性为主要特点的常染色体显性遗传病,随着息肉体积增大,患者年龄增加,消化系统及生殖系统等恶性肿瘤发病率明显增加,主要致病基因为19号染色体短臂上的LKB1/STK11(丝氨酸/苏氨酸激酶)基因,是一种肿瘤易感综合征,临床应及早处理胃肠道息肉及密切随访观察,预防恶性肿瘤的发生及早期诊治,减少PJS带来的危害。
Peutz-Jeghers syndrome (PJS) is an autosomal dominant disease characterized by mucocutaneous mucosal pigmentation, multiple gastrointestinal tract polyps, and familial heredity. As polyp volume increases, Patients with increased age, digestive system and reproductive system and other malignant tumors increased significantly, the main pathogenic gene on chromosome 19 short arm LKB1 / STK11 (serine / threonine kinase) gene, is a tumor-susceptible syndrome , Clinical treatment of gastrointestinal polyps should be early and close follow-up observation to prevent the occurrence of malignant tumors and early diagnosis and treatment to reduce the harm caused by PJS.