Objective To investigate the clinical manifestations in patients with 22q11.2 deletion syndrome (22q11.2DS) to improve the understanding of the disease.Methods Twenty patients with 22q11.2 DS were enrolled from Children's Hospital of Fudan University between August 2008 and April 2014.Cytogenetic and molecular genetic methods included fluorescence in situ hybridization (10 cases),and multiplex ligation-dependent probe amplification (10 cases).Age at the time of the diagnosis,sex and clinical manifestations were analyzed.Results The subject group consisted of 20 patients.Among them,13 cases (65％) were male and 7 cases (35％) were female.The median diagnostic age was 3.9 months.The presence of congenital heart diseases was identified in 17 patients (85％) and surgical correction was performed in 9 cases of them.The most frequent of complex congenital heart diseases were tetralogy of Fallot (20％) and pulmonary atresia (20％).Ten patients had varying degrees of T-cell immune function defects.Decrease in total lymphocytes and only CD8 counts were present in 45％ and 5％,respectively.Hypogammaglobulinemia was not detected in any patient.Six eases with T-cell immune function defects were treated with thymosin,4 of which were followed up for months,and the prognosis was good.Hypocalcemia was detected in 6 patients (30％),3 of whom presented with hypocalcemic seizures and hypoparathyroidism.Craniofacial dysmorphisms were detected in 3 patients(15％),2 of them only presented with micrognathia.Otorhinolaryngologic abnormalities were found in 4 cases (20％),3 of whom had laryngeal abnormalities,one of whom had cleft palate.Psychomotor developmental delay was found in 9 cases.Conclusions Congenital heart defects,hypocalcemia and/or impaired immune function are diagnostic features for 22q1 1.2 deletion syndrome,and they should be considered for cytogenetic analysis.

OBJECTIVETo evaluate the safety and feasibility of laparoscopic gastrectomy for gastric stump cancer.

METHODSClinical and follow-up data of 7 patients who underwent laparoscopic gastrectomy for gastric stump cancer in our department from January 2008 to July 2015 were analyzed retrospectively.

RESULTSThere were 5 male and 2 female patients, with a mean age of (62.1±10.7) years. Initial gastrectomy was performed for gastric cancer in 3 patients and peptic ulceration in 4. The initial surgery was B-II( gastrojejunostomy in 6 patients and Roux-en-Y gastrojejunostomy in 1. Duration between primary gastrectomy and occurrence of gastric stump cancer was ranged from 6-30 years for peptic ulceration, and from 11-15 years for gastric cancer. During the operation, adhesiolysis and exploration to locate the tumor were performed. Following total remnant gastrectomy and lymphadenectomy, intracorporeal anastomosis was accomplished by Roux-en-Y reconstruction. The methods of intracorporeal esophagojejunostomy were end-to-side approach using a circular stapler in 1 patient, side-to-side approach using an endoscopic linear staple in 2 patients, and hand-sewn technique in 4 patients. The operation time was (247.1±17.5) minutes and the intraoperative blood loss was (100.0±30.8) ml without transfusion. The number of retrieved lymph node was 19.1±4.8. The first flatus time, diet resumption time, postoperative hospital stay were (3.3±1.5) days, (3.7±0.8) days, (9.4±2.6) days, respectively. One patient experienced gastrointestinal bleeding that was managed conservatively and ultimately cured. Seven patients were followed up till January 2016. After follow-up from 6 to 38 months, 1 patient died of peritoneal metastasis 17 months after surgery, and 1 patient died of Alzheimer's disease 19 months after surgery. The other 5 patients were still alive without metastasis or recurrence.

CONCLUSIONLaparoscopic gastrectomy for gastric stump cancer is feasible and safe.

Aged ; Anastomosis, Roux-en-Y ; Blood Loss, Surgical ; Female ; Gastrectomy ; Gastric Bypass ; Gastric Stump ; pathology ; surgery ; Humans ; Laparoscopy ; Length of Stay ; Lymph Node Excision ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Operative Time ; Retrospective Studies ; Stomach Neoplasms ; surgery ; Surgical Stapling

OBJECTIVETo evaluate the feasibility and safety of laparoscopic pancreaticoduodenectomy(LPD).

METHODSData of 66 patients from Sir Run Run Shaw Hospital undergoing LPD from September 2012 to September 2014 were reviewed. There were 44 male and 22 female with the mean age of (58.7±10.3) years and mean body mass index of (23.5±3.9)kg/m(2). Forty-five patients presented the symptoms and four of all had the history of abdominal surgery, including 2 cases of laparoscopic pancreatic surgery.

RESULTSOf 66 patients underwent laparoscopic procedure, 1 patient underwent LPD combined with right hepatic resection, 1 patient underwent laparoscopic distal gastrectomy with LPD, and 1 patient underwent LPD after laparoscopic distal pancreatectomy. The mean operative time was (367±49) minutes. The mean blood loss was(193±126)ml. The rate of overall postoperative complications was 36.4%(24/66), with 4.5%(3/66) of B or C pancreatic fistula and 7.6% (5/66) of bleeding. The mean postoperative hospital stay was (18.9±12.1) days. Mean tumor size was (3.8±2.3) cm, and the mean number of lymph nodes harvested was (20.3±10.9). Forty-severn patients were diagnosed as pancreatic adenocarcinoma(n=18), cholangiocarcinoma(n=7), ampullary adenocarcinoma(n=21), and gastric cancer(n=1), respectively.

CONCLUSIONLPD is feasible and safe under the skilled hand.

Adenocarcinoma ; surgery ; Aged ; Anastomosis, Surgical ; Female ; Humans ; Laparoscopy ; Length of Stay ; Male ; Middle Aged ; Operative Time ; Pancreatectomy ; Pancreatic Fistula ; pathology ; Pancreatic Neoplasms ; surgery ; Pancreaticoduodenectomy ; Postoperative Complications

Objective: To summary the experience of 233 cases of laparoscopic pancreaticoduodenectomy (LPD) performed by a single surgical team. Methods: Data of patients undergoing LPD from September 2012 to October 2016 were reviewed. There were 145 males and 88 females with the mean age of(60.3±13.0)years old, ranging from 19 to 92 years old, and the mean body mass index of (22.8±3.5)kg/m^2, ranging from 16.3 to 36.8 kg/m². There were 195 patients with clinical manifestation and 54 patients who had the history of abdominal surgery. Results: LPD were performed on 233 patients by same surgical team consecutively. The mean operative time was(368.0±57.4)minutes. Mean blood loss was(203.8±138.6)ml. The postoperative morbidity rate was 33.5%, with 6.9% of grade B or C pancreatic fistula and 9.9% of bleeding. The reoperation rate was 5.6%. The mortality during 30 days after operation was 0.9%. Mean postoperative hospital stay was (18.1±11.2)days. Mean tumor size was (3.9±2.4)cm, and the mean number of lymph nodes harvested was 21.3±11.9.One hundred and sixty-three patients were diagnosed as malignant tumor, including pancreatic adenocarcinoma(n=84), cholangiocarcinoma(n=17), ampullary adenocarcinoma(n=55), duodenal adenocarcinoma(n=5), gastric cancer(n=1)and duel cancer (n=1) located in distal stomach and duodenum. Conclusion The key point to make laparoscopic pancreaticduodenectomy a routine safe procedure is to operate the procedure under skilled hands in selected patients via suitable surgical approaches.

Objective To evaluate the specific antibody-producing capacity of locally infiltrating B lymphocytes in lesions of patients with pemphigus vulgaris (PV).Methods Totally,35 patients with PV and 22 healthy controls were enrolled into this study.Skin tissues were resected from blisters or erosions of the patients with PV,and from normal skin of healthy controls.Then,mononuclear cells were isolated from these skin tissues.Flow cytometry was performed to determine the percentages of lymphocytes,CD 19+ B lymphocytes,and desmoglein (Dsg)1-and Dsg3-specific CD19+ B lymphocytes.B lymphocytes isolated from the lesional skin of patients with PV were cultured in vitro.Enzyme-linked immunosorbent assay (ELISA) was conducted to determine titers of anti-Dsg1 and anti-Dsg3 antibodies in the cell culture supernatant.Receiver operating characteristic (ROC) curve analysis was conducted to calculate positive rates of anti-Dsg1 and anti-Dsg3 antibodies.Results The percentages of lymphocytes (17.95％ ± 3.85％) and CD19+ B lymphocytes (4.27％ ± 1.13％) were significantly higher in the lesional skin of PV patients than in the normal skin of healthy controls (7.83％ ± 1.29％,0.61％ ± 0.31％ respectively;t =2.49,U =13.00 respectively,both P ＜ 0.05).Among the CD19+ B lymphocytes in the lesional skin of PV patients,the percentage of CD19qgG+ B cells was (38.33 ± 5.56)％,and percentages of Dsg1-and Dsg3-specific CD19+ B lymphocytes were 12.87％ ± 1.267％ and 10.42％ ± 1.243％ respectively.After the in vitro culture for 6 days,the titers of anti-Dsg1 and anti-Dsg3 antibodies in the cell culture supematant were (4.89 ± 1.56) U/ml and (35.45 ± 13.03) U/ml respectively,with their positive rates being 85％ (17/20)and 95％ (19/20) respectively.Conclusion There are Dsg1-and Dsg3-specific B lymphocytes aggregating in the lesional skin of patients with PV,which can produce anti-Dsg1 and anti-Dsg3 antibodies after in vitro culture.

Objective: To evaluate the safety and feasibility of laparoscopic radical antegrade modular pancreatosplenectomy(Lap-RAMPS) for left-sided pancreatic adenocarcinoma. Methods: Clinical data of total 12 patients underwent Lap-RAMPS for left-sided pancreatic adenocarcinoma at Department of Gastrointestinal and Pancreatic Surgery, Zhejiang Provincial People′s Hospital from March 2016 to August 2017 were reviewed retrospectively.There were 7 male patients and 5 female patients, with median age of 60.5 years old(47-68 years old). Abdominal enhanced CT, pancreatic MRI, PET-CT were performed on all patients to evaluate the lesion and exclude metastasis.Follow-up were done with out-patient clinic or telephone consultancy until October 2017. Results: All patients underwent pure Lap-RAMPS.The medium operative time was 250 minutes(180-445 minutes), and the blood loss was 150 ml(50-500 ml). The medium first flatus time and diet resumption time were 3.0 days(1-5 days) and 3.5 days(1-7 days) respectively.The medium postoperative hospital stay was 9 days(4-18 days). Morbidity occurred in 8 patients with gastric empty delay(n=1), bleeding(n=1), fluid collection(n=3). There was no mortality.The medium overall number of retrived lymph nodes was 15.6 and the positive rate was 41.7%. The R0 rate was 100%.The medium follow-up was 10 months.One patient was diagnosed as liver metastasis after 8 months and accepted chemotherapy.One patient died after 14 months for tumor recurrence and metastasis.Others survived without tumor recurrence or metasitasis. Conclusion Lap-RAMPS is safe and feasible with accepted oncological outcomes for selected left side pancreatic adenocarcinoma under skilled hands.

Objective To evaluate the safety and feasible of adjacent organ resection during laparoscopic pancreaticoduodenectomy(LPD),and summary the surgical strategies.Methods Clinical data of 15 adjacent organ resections combined with LPD from March 2013 to September 2017 were reviewed.There were 10 male and 5 female patients aging from 20 to 86 years,and the body mass index ranged from 19.6 to 34.5 kg/m2.Two patients had previous abdominal surgical history.Two patients underwent preoperative chemotherapy.Results The resected adjacent organs included liver (n =4),stomach (n =3),colon(n =6),right kidney with embolectomy and vasoplastic of inferior vena cava (n =1),and spleen artery aneurysms(n =1).The operative time ranged from 280 to 450 minutes,and the blood loss ranged from 100 to 450 ml.The total complication rate was 5/15 and no one died in 90 days after surgery.The postoperative hospital stay ranged from 10 to 42 days with medium 18 days.The pathology included adenocarcinoma of stomach and duodenum (n =1),gastric cancer invading pancreas or duodenum (n=2),ampullary adenocarcinoma with left hepatolithiasis(n=1),ampullary adenocarcinoma with a benign lesion in left liver (n =1),ampullary adenocarcinoma with single liver metastasis (n =1),ampullary adenocarcinoma(n =1),pancreatic intraductal papillary mucinous neoplasm with splenic artery aneurysms (n=1),pancreatic neuroendocrine neoplasm with colon cancer (n =1),distal common bile duct adenocarcinoma involving righ hepatic duct (n =1),pancreatic neuroendocrine neoplasm invading inferior vena cava and right renal vein(n=1),duodenal adnocarcinoma(n =1),duodenal ewing's sarcoma(n =1),duodenal intesititialoma (n =2).The follow-up was from 3 to 40 months with the medium survival of 17.5 months.Conclusions The oncological outcomes of PD combined with adjacent organ resection is acceptable.Surgical treatment for those patients with periampullary neoplasma and adjacent organ lesions should be aggressive.

Objective To evaluate the safety and feasible of adjacent organ resection during laparoscopic pancreaticoduodenectomy(LPD),and summary the surgical strategies.Methods Clinical data of 15 adjacent organ resections combined with LPD from March 2013 to September 2017 were reviewed.There were 10 male and 5 female patients aging from 20 to 86 years,and the body mass index ranged from 19.6 to 34.5 kg/m2.Two patients had previous abdominal surgical history.Two patients underwent preoperative chemotherapy.Results The resected adjacent organs included liver (n =4),stomach (n =3),colon(n =6),right kidney with embolectomy and vasoplastic of inferior vena cava (n =1),and spleen artery aneurysms(n =1).The operative time ranged from 280 to 450 minutes,and the blood loss ranged from 100 to 450 ml.The total complication rate was 5/15 and no one died in 90 days after surgery.The postoperative hospital stay ranged from 10 to 42 days with medium 18 days.The pathology included adenocarcinoma of stomach and duodenum (n =1),gastric cancer invading pancreas or duodenum (n=2),ampullary adenocarcinoma with left hepatolithiasis(n=1),ampullary adenocarcinoma with a benign lesion in left liver (n =1),ampullary adenocarcinoma with single liver metastasis (n =1),ampullary adenocarcinoma(n =1),pancreatic intraductal papillary mucinous neoplasm with splenic artery aneurysms (n=1),pancreatic neuroendocrine neoplasm with colon cancer (n =1),distal common bile duct adenocarcinoma involving righ hepatic duct (n =1),pancreatic neuroendocrine neoplasm invading inferior vena cava and right renal vein(n=1),duodenal adnocarcinoma(n =1),duodenal ewing's sarcoma(n =1),duodenal intesititialoma (n =2).The follow-up was from 3 to 40 months with the medium survival of 17.5 months.Conclusions The oncological outcomes of PD combined with adjacent organ resection is acceptable.Surgical treatment for those patients with periampullary neoplasma and adjacent organ lesions should be aggressive.

As research delves deeper into the mechanisms of tumor immune responses,studies reveal the importance of microbial com-munities within the tumor microenvironment in tumor progression and their interactions with the host immune system.Intratumoral micro-biota could influence the tumor microenvironment,thereby promoting or inhibiting tumor growth and development.Despite this import-ance,the specific role of intratumoral microbiota impacting cancer immunotherapeutic efficacy remains largely unexplored.A deeper under-standing of the characteristics and biological functions of tumor-specific microbiota heralds a potential revolutionary innovation in cancer treatment.In this review,we introduce the discovery and sources of intratumoral microbiota,also addressing its composition,and discuss tumor tissue characteristics.Moreover,we briefly review the history of cancer immunotherapy development with a particular focus on the research progress concerning the impact of intratumoral microbiota on cancer immunotherapy.Furthermore,we explore emerging strategies that combine targeting intratumoral microbiota with immunotherapy to enhance immune efficacy,inhibit tumor progression,and improve cure rates,anticipating that this approach could represent a new direction for enhancing treatment outcomes and prospects.