Clinical analysis of 31 cases of Pcutz-Jeghers syndrome / 中国综合临床

ABSTRACT

Objective To investigate the clinical features,complications,treatment and prognosis of patients with Pcutz-Jeghers syndrome.Methods The clinical data of thirty-one cases of Pcutz-Jeghers syndrome treated in the First Hospital of Shanxi Medical University from January 1998 to December 2016 was analyzed retrospectively.Results There were11 cases with definite family history(35%,11/31).There were 21 cases of mucocutaneous pigmentation(67.7%,21/31),31 cases of gastrointestinal polyps(100%,31/31), including 8 cases of canceration(25.0%,8/31),24 cases of digestive tract bleeding(77.4%,24/31),20 cases of intussusception(64%,20/31),and 1 case of intestinal perforation(3.2%,1/31).6 cases were treated with endoscopic high frequency electrocoagulation,12 cases received polypectomy,17 cases underwent resection of intestinal tract.3 patients with intractable intussusception underwent plication of small intestine,and 4 patients with colon cancer underwent total colectomy combined with lymph node dissection.Conclusion Peutz-Jeghers syndrome often induce intractable intestinal obstruction and gastrointestinal tumor,resection of intestinal lesions should be favorable.For small intractable intussusception,the plication of small intestine has certain value; for its potential malignancy,it should be strictly monitored and actively treated.Gene therapy and drug therapy will guide for the future development.