RÉSUMÉ
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.
RÉSUMÉ
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.
RÉSUMÉ
Objective To study the growth effect of human cholangiocarcinoma cell line QBC939 treated by norcantharidin (NCTD) and preliminary illustrate the potential mechanism.Methods The human cholangiocarcinoma cell line QBC939 was detected by MTT assay,flow cytometry,immunocytochemistry after the treatment of NCTD in vitro.Results NCTD displayed inhibitory effect on growth of QBC939 from different doses of 0.125,0.75,2.5,10,120 μg/ml after 48 h (P <0.05).It was in a dose and time dependent manner.Dose-effect curve was drawn and IC50 value was (3.66±1.14) μg/ml.The flow cytometric profiles showed that the rate of cell apoptosis enhanced following increasing the concentration of NCTD[(8.6±0.4) %,(17.6±0.3) %,(22.9±0.4) %,(25.5±0.9) % and (31.1±1.5) %,respectively]and cells blocked in the G2/M phase after treatment with 2.5 μg/ml NCTD[(14.1±1.0) % and (5.7±0.3) %].The expression of the protein caspase-3 elevated after different concentrations of NCTD co-cultured with QBC939 compare with contrast group.Conclusion NCTD has an inhibitory effect on proliferation of QBC939 cell line,and the mechanism might be related to the induction of cell apoptosis and blockade of cell cycle.