The Study Of Therapy And Associated Gene Expression Of Peutz-Jeghers Syndroms

Objective TO study the optimal standard of diapnosis,follow uP, and principle of surgery treatment. Metbods RetrOspectiveanalysis of 27 cases from l984 to 2000 in our hospital and l88 cases inChinese literature of recent l0 years. Re8uIt About 55.6% (l5/27)have family histOry, the mean age is l5.l(3~34) fOr onset of PJS and is3.9 for the aPpearance of melanin pigmentation. The cancer incidenceamong the 27 cases is l8.3%. In our 27 cases, 487 gastric and colonicpolyps were resected by preoperative endoscopy, and the biggest one inrectal is l0 X 8 X 6 and divided into l l8 pieces fOr rescetion. 3l2 polyPsof small bowel were resected by intraoperative endoscopy The highestnumber removed one time is 60 polyps. All the operations are result in"clean intestinal". Among the 27 cases, 5 had adenom' 3 had cancer ofsmall bowel, 2had colorectal cancer. 2 cases bled after operation andrecovered by conserv8tive management. l patient died of perfOration. lchild patien reoperation 42 months late. Among the 188 cases, the meanage of onset is 26.3 years and 42.0% have boly histOry. The cancerincidence is l8%(34ll88) and there are 25 cases have @pearant PJSpedigree. The onset age of cancer is from l5 tO 66 years. CouclusionPJS patients have different phenotyPe. The emergency oper8tion andidentification lurninal polyPs by palpation are not effective methOds.Intraoper8tion endoscopy is an effective method to tre8t PJ polyPs. If thepolyP is larger than l .5cm, laParOtomy should be considered. Patielltswho are over 30 years or have family history have high risk of cancer...