| 曹强坚李威.黑斑息肉综合征临床诊治进展[J].,2011,11(11):2190-2193 |
| 黑斑息肉综合征临床诊治进展 |
| Advances in Diagnosis and Treatment of Peutz-Jeghers Syndrome |
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| DOI： |
| 中文关键词: 黑斑息肉综合征 诊断 治疗 进展 |
| 英文关键词: Peutz-Jeghers syndrome Diagnosis Treatment Advances |
| 基金项目: |
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| 中文摘要: |
| 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。 |
| 英文摘要: |
| Peutz-Jeghers syndrome (PJS), an autosomal dominant inherited disorder, is characterized by polyps throughout the
gastrointestinal tract and mucocutaneous pigmentation. PJS patients have a significantly increased risk for developing malignant tumors
of multiple organs both in the gastrointestinal tract and in extragastrointestinal sites. The mutations in the serine/threonine kinase 11
(STK11) gene at 19p13.3 have been demonstrated to be responsible for most PJS cases. It is a tumor susceptibility syndrome. Therefore,
to prevent and early diagnosis the related cancers in PJS patients, it should be treated the polyps early and set a scientific follow-up schedule
in clinic, reducing the harm to a large degree. |
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